Ava Szajnuk’s Journey
Ava Szajnuk’s Journey
Born: June 4, 2007
Saved on June 15, 2013
Saved again on June 26, 2013
Saved again on July 3, 2013
Saved again on July 12, 2013
2019: “Push Through” by Ava Szajnuk
2013 – 2015: Ava’s Journal by Gina Szajnuk
Ava was born with an arachnoid cyst. We discovered it when she was three years old. At five years old, we were told that Ava has a genetic dysfunction, an autonomic neuropathy, small fiber polyneuropathy. Ava has been seen at over seven hospitals and over fifty specialists. Her medical record is over seven hundred pages. She suffers pain daily. At this time, there is no treatment for Ava’s pain. In other words, Ava lives in the world of the unknown.
Ava’s Szajnuk’s Journey started before my first entry but this is a good place to start…
February 3, 2013:
I wanted to send an update to all of you. Thank you for your concern for Ava as well as for me. It’s hard to talk about it in front of Ava right now. I thought this email might help everyone understand what is happening. I am trying to understand what is happening myself so bare with me. Please know that this is all way over my head. I’m going to try to explain what is happening as how I understand it today.
Dr. Sarah Chamlin is one of the top Dermatologists in the city. We had an appointment on Friday morning with her. She requested that I write a medical journal about Ava. She was sent the journal as well as the pictures this morning. She actually called me today at 7:30a (Sunday) to discuss Ava’s case.
Here is an update:
They are bringing in the top two doctors at Ann & Robert H. Lurie Children’s Hospital of Chicago. Dr. Nancy Kuntz is a Neurologist that is familiar with Erythromelalgia. They are also bringing in the doctor that they call “House”. He’s like the character on the TV show. He is brought in when no one knows what is going on. His name is Dr. Robert Listernick. He is known for Consultative Pediatrics, children with difficult diagnostic problems. Ava is a very rare case. Erythromelalgia is a “finding” it is not a diagnosis. Erythromelalagia does not belong to just one department at the hospital. They are all working together. They do not know if it is a Primary condition or a Secondary condition. Primary means it is something genetic. They want to test her for a certain gene mutation this week. There is something about a sodium channel mutation that was discussed as well.
“If the Erythromelalgia is a Secondary condition, it is unknown as to what it is or going to be. Symptoms of Erythromelalgia preceded the onset of a myeloproliferative disease by a mediam of 2 1/2 years. Myeloproliferative diseases are defined as the following: Myeloproliferative diseases (MPDs) are a heterogenous group of disorders characterized by cellular proliferation of one or more hematologic cell lines in the peripheral blood, distinct from acute leukemia. According to the French-American-British (FAB) classification, chronic myeloproliferative diseases consist of 4 diseases: chronic myelogenous leukemia (CML); polycythemia vera (PV); essential thrombocythemia (ET); and agnogenic myeloid metaplasi (AMM), which is also known as myelofibrosis (MF). In 2002, the World Health Organization (WHO) proposed an alternate classification schema for these diseases, adding chronic neutrophilic leukemia (CNL) and chronic eosinophilic leukemia.”
Dr. Chamlin discussed “flushing” as well as Erythromelalgia. I need to research what “flushing” means this afternoon. They want to do an abdominal MRI as well as an MRI of her kidneys. She is already scheduled for the MRI of her brain to make sure that her arachnoid cyst has not gotten bigger. The Rheumatologist did a ton of blood work in December but did not do an updated CBC. They want to do that this week as well.
Friday night was the worst night with Ava since this all started. She had a lot of bruises all over her body. We decided to take her off of aspirin until the three doctors talk tomorrow. She is now complaining that her head hurts. The last two nights she has had headaches.
The good news is that she usually wakes up happy and rested. We seem to start the cycle all over every day in the early evenings.
She has a lot of anxiety right now. She’s trying to process everything. They are going to set her up with a “Life Coach” this week. Dr. Chamlin said I should spend as much time as possible with her right now. I’m not sure how to interpret that comment. So, please pray for Ava. Let’s just hope this is all something not serious.
However, please understand that we are very worried and are trying to process all of this along with her.
Thank you again for all of your love and support. I will keep you updated as much as I can this week. I appreciate all of the phone
calls and texts. Please understand if I cannot talk when you reach out to me.
March 20, 2013
I thought it was time to update you on Ava. We are still waiting for the results of the gene test. We have been waiting since February 5th. Hopefully, we will have the results soon. However, we have a feeling that it will come back negative. The gene test was for Primary Erythromelalgia. They have listed Erythromelalgia now as a “finding” not as a diagnosis.
The Mayo Clinic listed Dysautonomia as the number one possibility. Dysautonomia is an umbrella medical term utilized for a group of complex conditions that are caused by a dysfunction of the autonomic nervous system (ANS). The ANS regulates all of the unconscious functions of the body, including the cardiovascular system, gastrointestinal system, metabolic system, and endocrine system. A dysfunction of the ANS can cause debilitating symptoms and may pose significant challenges for effective medical treatment.
Ava started Clonidine at night a few weeks ago. Unfortunately, it doesn’t seem to be helping. We are now starting it twice a day to see if it will help. She has a lot more symptoms. The doctor said that Ava is still presenting with new symptoms. I am journaling every day and taking pictures every day. I recently went back and looked at Ava’s baby pictures. There were a lot of pictures that looked like she has had this since she was a baby.
We have also started to notice similar symptoms with Oskar and Lucy. We are taking pictures daily and journaling the two of them as well. Justin and I both feel that there is a strong possibility that all three children have the same thing.
Justin spoke with Dr. Kuntz’s nurse yesterday. Joan went over the upcoming schedule with us. Ava is scheduled to have her MRI on April 4th. This is her yearly MRI to monitor her Arachnoid Cyst. Dr. Kuntz wants to review it as well. We are doing a 24 hour urine test this week to test for toxins. We want to rule out that this is being caused by anything in the environment. Dr. Kuntz is going to meet with us after her MRI. She is also going to meet Oskar and Lucy.
If you have any questions, please let me know. I truly appreciate your support, concern and prayers. Thank you for being patient with me and ALL of my anxiety.
March 21, 2013: Ava took her medicine in the morning and then went to school today. She had a great time. She came home and would not get out of the car. She felt really tired. She slumped over during lunch. She wouldn’t eat anything. She ate a potato pancake. She went into her room and wanted to rest. We offered to play games, read, etc. She didn’t want to do anything. In the early evening, Ava’s hands were really raw and were hurting. I tried the organic ointment I bought yesterday. It made her hands look the worst they have in a long time. Bright red. She was in a lot of pain. We tried to wash the ointment off with water. She sat in bed with her arms up for the rest of the night.
March 22, 2013: We went to a movie today. Ava was exhausted after the movie. She came home and rested in her room again. Her hands were raw all day. We tried Vaseline today. It made the hands really red. They were swollen too. Her butt, legs and bottom of her feet were all bright red tonight. She didn’t really well. She was really worried about dying last night. She was scared and wanted to talk about it. She asked why her hands always hurt.
March 23, 2013: Ava’s hands are still really raw today. We changed our water system in our house yesterday. We are hoping the bath/shower water will be less harsh for the kids. She doesn’t want to put anything on her hands this morning. She is scared. We took the kids for a drive for 30 minutes for lunch. Ava fell asleep. We couldn’t wake her up. We ordered the food to go. She was upset when we got home. She wanted to know why she is always sick. She was freezing and her hands/arms were very cold. Her hands are really raw still today. We just played golf and tennis inside the house to cheer her up. Now she says her tummy hurts and she feels like she’s going to throw up. She is resting now.
March 24, 2013: Ava was hyper all day with the family. She was in pain that night. Her hands and arms were really red after everyone left. It seems like she doesn’t feel her hands getting redder until she starts to calm down after being hyper.
March 25, 2013: Her hands/arms were really dry and red. We had a low-key day today.
March 26, 2013: Ava’s feet got the same weird rash on them today. She took a bath for a long time today. Grandma gave her the bath.
March 27, 2013: Ava had the same circle rash on her feet today. It happened after only 30 seconds in the warm bath water. I got her out right away. Her hands were raw again this morning. We put Aquaphor on it again this morning. She took her Chlonidine this morning as well. I took her to get the urine test and blood test done here in Madison. It was for ruling out Mast Cell Disease. Wow. It’s really scary. I’m praying that she doesn’t have it. There are so many triggers for Mast Cells. Our lives would change drastically. Mast cells can take over your body and eventually kill you. Triggers make more Mast Cells produce. She would have to avoid all triggers. The blood and urine were sent to the Mayo Clinic. I’m sure it’s going to take a long time for the results. Her hands got really red as did her arms, face, elbows and feet today when she was waiting at the lab. She didn’t cry very much. I was really proud of her. She did the blood test without any drama. They gave her a teddy bear. I took her to pick out something in the gift shop too. We went out for ice cream and hot dogs after the hospital. I promised her that we will always do something fun when we have to do something scary. I took her through the University of Wisconsin-Madison campus. I told her that this was where Grandma, Uncle Shannon, Aunt Chrissy and I all went to school. We went to college there. She said, “I don’t think I will grow up and go to college. I’m not going to grow up because of my hands.” It broke my heart. It’s a hard day today. I’m scared and want answers.
March 30, 2013
As we updated you previously, the Mayo Clinic diagnosed Ava with an Autonomic Neuropathy, which is an umbrella medical term utilized for a group of complex conditions that are caused by a dysfunction of the autonomic nervous system (ANS). They listed Dysautonomia as the “number one most likely.”
The Children’s Hospital in Chicago has her current diagnosis as Erythromelalgia. We were waiting to find out if it was primary or secondary. We received the call last night that the test came back negative for Primary Erythromelalgia. It is good news. However, Secondary Erythromelalgia is now back on the table. This is not good news. As I explained in my first update to you in February, Secondary Erythromelalgia is a precursor to some other underlying conditions.
We also have Mast Cell Disease on the table now. At the end of February, Ava had an unusual redness on her palms and feet after we swam in a warm pool in the Wisconsin Dells. We took her to the ER in Madison. They would not touch her. They were not familiar with Erythromelalgia. They called her doctor in Chicago. She saw us the next day. By then, the redness was gone. We showed her Ava’s pictures. She gave us an order for lab work for the next time she had the same outbreak. Unfortunately last Tuesday, Ava had the same redness on her feet and palms after her bath. She had it again on Wednesday morning after only about 30 seconds in the warm bath water. This outbreak is different than her daily redness/swelling. The lab work done on Wednesday was to rule out Mast Cell Disease. We are now waiting for those results. They told us we should get them back in about 7 to 10 days.
Ava is scheduled to have her Cranial MRI this Thursday per her neurosurgeon. It is a follow-up for her arachnoid cyst. However, her current neurologist would like to see it as well. We are concerned about Ava going under sedation for the MRI. Her MRI is being done at a different hospital. I called their Head of Anesthesia to inform her of Ava’s current possible diagnosis. She had never heard of Erythromelalgia. She called Ava’s doctor at Children’s to discuss the risks. They both feel confident that we are not putting Ava in any danger with sedation. I spent a few hours researching the risks of anesthesia/mast cells risks as well as anesthesia/autonomic neuropathies risks recently. There is a lot of research that supports the risks. As a parent, you want to do what is best for your child. However, our doctors feel strongly that they need Ava to have her MRI. Her neurosurgeon wants it done. Her neurologist wants it done. My anxiety goes off the charts when I have to put my children under sedation. It’s not something that I take lightly. Ava has been sedated now three times. She was previously sedated for two MRIs. She handled them just fine. However, she was sedated for her dental surgery in August. She was given Fentanyl as well as Codeine. She was not the same after her surgery. For example, she was confused, dyslexic, manic, silly and had short-term memory loss. Codeine is on the list as a definite trigger for mast cell degranulation. We are worried that this might have played a part in what is going on with her now. The list for mast cell triggers is very long. We already know that the following are triggers for her as of now: cold weather, hot weather, emotion/stress, physical activities, warm water and cold water.
We are also still waiting for the results on Ava’s 24-hour urine test for toxins. We should get them this week as well.
April 9, 2013
I’ve been asked by a few family members for an update. We have had a lot of information to process since Friday afternoon.
So, here we go…..
-The Heavy Metal results came back normal. They specifically mentioned that her Mercury level was within the normal range. Good News! We are waiting to get an actual copy of the report. We want to know all of the metals that were tested.
-The Mast Cell Disease results came back from The Mayo Clinic. It was normal too. There is always a small chance that it was a false negative. However, we are very relieved that it was negative. Great News!
-Ava had her MRI yesterday. My dad looked at the images to compare them with her previous MRI in 2011. He felt it looked the same. The report said everything is stable and the cyst is the same size. It’s still very large…but Great News! We will still be meeting with her Neurosurgeon at the end of the month.
-I spoke with Dr. Kuntz on Saturday afternoon. We feel that we are at a roadblock with her at this time. She wants to continue to treat Ava with Clonidine. We do not feel like Clonidine is helping her. She doesn’t have a lot more to offer us right now. She wants to continue treating Ava’s symptoms. She stressed again how hard Ava is to diagnose. She still uses the word “unknown” over and over. I explained to her that Ava’s skin is getting worse every week. Since she is a Neurologist, she wanted us to follow up with Dermatology. We still have an appointment set with her at the end of the month.
Ava’s symptoms change weekly. She has had leg pain in her shins. At times, the pain makes her cry. She has leg pain in her thighs as well. We are hoping that they are just growing pains and not related to anything else. Occasionally, she itches all over. Her hands, arms, elbows, knees, arms, legs, lips, cheeks, feet etc.….all still get really red when she is triggered. She used to just get mottled on her hands/arms. Now she mottles all over her entire body. She doesn’t like to eat anything. Her tummy hurts when she eats. She is tired every day. She has good hours of activity….then followed by exhaustion. She takes a lot of naps now. We need answers. We have had wonderful news with the negative test results. However, we must keep fighting for answers.
We also feel that Ava’s hands have gotten a lot worse in the last week. She played outside one afternoon and her skin started to pull apart. We feel that her skin is eroding away on the top of her hands. I put a call into The Mayo Clinic Dermatology Department on Friday night. They called back right away. The doctor is not the one that saw her during our trip there. However, she was very opinionated. She stressed the need for us to continue to try to find out what is causing her Autonomic Dysfunction. She explained again that Erythromelalgia is only a “finding”. We need to treat Ava’s skin as a dermatological problem but to continue to fight for answers on the bigger scale of things. She was very helpful and made me feel better. She said to expect a call from Dr. Tolefson early next week. Dr. Tolefson was one of the dermatologists that we met with during our visit.
Dr. Tolefson called this morning. She hadn’t heard yet that the gene test came back negative for Primary Erythromelalgia. She said she had felt it was going to come back negative. The Mayo Clinic really thinks differently towards Erythromelalgia than the Children’s hospital in Chicago. They do not want to accept it as a diagnosis. They definitely feel that Ava has an Autonomic Dysfunction (also known as Dysautonomia) and they want to find out why.
Dr. Telefson and I discussed Ava’s skin. I had researched the connection between Autonomic Dysfunction and dry skin. I found the diagnosis of Atopic Dermatitis. She said that dermatitis is very common. She said that we can treat Ava’s skin problem as a separate issue right now and see if it improves. However, she strongly feels that it is coming from the larger issue of her Autonomic Dysfunction. She did prescribe something stronger for her skin. We will try it tonight. Ava’s been sleeping all afternoon.
Moving forward, Dr. Tolefson is going to reach out to the Head of the Dermatology Department, Dr. Mark Davis. He was the main reason that we wanted to go to The Mayo Clinic. She is also going to reach out to the Neurology Department there as well. We discussed the other two kids. I explained how I’ve been trying to find out why Oskar is always so tired for over a year now. He falls asleep when he eats. He turns red like Ava at night. She felt he should be evaluated as well. I asked if there could be a connection with all three of my children and an Autonomic Neuropathy. She said absolutely. It’s all in the genes. She explained that they have a Pediatric Diagnostic and Referral Clinic there. She is going to send me the information as to how to get Oskar evaluated. I asked her how we got in to the clinic without going through the evaluation clinic. She had no idea. So, I guess that was why we were only there for the one appointment. We got in the back door. She said we were very fortunate. Ava is their patient now. They will help us find more answers. Great news too!
Thank you for taking the time out of your busy day to read our update.
Thank you again for your prayers, support, friendship and love.
May 3, 2013:
We wanted to update you on our recent visit to the Mayo Clinic. We took both Ava and Oskar for an evaluation this week with their Pediatric Evaluation and Referral Clinic. We met with the Head of the Pediatric Department, Dr. Phil Fischer. He is their Autonomic Specialist there at the hospital. The kids responded very well to him. We met with him on Monday morning. We had a lot of blood work done on Tuesday. We had a follow up meeting with him on Wednesday. We are currently waiting for several results.
Here is a breakdown of what we do know this far from our time at the Mayo Clinic. Dr. Fischer explained it to us in a way that was much easier to understand than previously discussed. Both Ava and Oskar have an Autonomic Neuropathy. They are fascinating to doctors because they are pushing the edge of science right now. The Mayo Clinic has not previously seen children like them. They have seen babies born with Familial Autonomic Neuropathy that are very, very sick. They have seen teenagers with Dysautonomia POTS. Ava and Oskar are obviously in between those two specific diagnoses. He said they are in the “unknown area” between being completely healthy on one end and having a disease on the other end.
They explained it to us that Ava and Oskar have difficulty regulating their temperature. When you have a hard time regulating your temperature, you have a hard time regulating your blood flow. When you have a hard time regulating your blood flow, your entire body is affected. For Ava, triggers such as cold/warm water, cold/warm weather, emotions/stress and physical activity all affect her through her faulty thermostat. Both of their digestive systems are affected as well. At times, Oskar actually falls asleep when he eats dinner. Neither of the kids ever want to eat or eat very much. Oskar has more fatigue, low-grade fevers and often shuts down when over stimulated. The kids have very heightened senses. We always noticed the kids get overwhelmed when there are a lot of people around or it’s really loud. For example, we took Oskar to the circus and the planetarium. He fell asleep during both outings. It’s his body’s way of handling too much noise. Both kids get very excitable. They usually become very fatigued after a lot of excitement. Ava and Oskar show different symptoms but it comes back to the same overall issue.
He explained the reason for Ava’s redness. When a body is hot or active, the blood flow comes to the top of the skin. The heat wants to get out. When a body is cold, the heat moves inward to protect the organs. Her limbs become very cold and she gets tired. Oskar gets red as well. At this time, he does not get as red as often as Ava. The Mayo Clinic has even taken Erythromelalgia off the table for now for Ava.
Both of the kids were low in iron. This is not the problem but it most definitely aggravates an Autonomic Neuropathy. We are giving them iron supplements now. It should hopefully help with the fatigue.
One of the test results that we are waiting for have to do again with Mast Cell Disease, which they think is unlikely. They are also checking for several other very rare conditions. I will update you on them if the results are positive. They are pretty complicated. If you are interested, let me know. I will explain them to you.
We also believe that Lucy will be diagnosed with an Autonomic Neuropathy as well. She has similar symptoms to both Ava and Oskar. She is also low on iron. They said they will see her once they get a better understanding of what’s going on with Ava and Oskar. They didn’t want to put her through all of the tests yet since she is so young.
Dr. Fischer made us feel a lot better. He validated many things for me, which made me feel much better. It helped give us a better outlook on everything. No matter what the results of the tests are, Mayo plans to be there for us. They told us that we are now part of the Mayo Family. They will help us come up with a plan to minimize triggers and to help deal with their stomach problems. Best case scenario, the kids will be followed by them by having a trip up to Mayo every summer to have a full workup to make sure nothing else starts happening.
May 3, 2013: Ava hit her head on the coffee table tonight. We were out to dinner. She was playing with the babysitter, Lizette. They were throwing pillows up into the air. She said she lost her sight for a second. She fell down and hit the edge of the table along her jaw line on the left side of her face. The bruise was really dark and in a straight line from the edge of her lip all the way to her ear. We iced it that night.
May 4, 2013: Ava’s head hurt all day. She slept four hours that afternoon. I called Dr. Ruge’s service to see what I should do. He called right away. He said that as long as she wasn’t vomiting, then we should be ok.
May 5, 2013: Her head hurt really badly. I called her Dr. Ruge’s service again today. Again, he said that as long as she wasn’t throwing up that we should be ok. However, if we felt we should take her to the ER at Lutheran General, he would order a CT scan. We went to the ER. We arrived at 7:19p. They sent us home without the CT. Her head was still hurting. All they gave us was Tylenol. We were out of there by 8:43p. Differential diagnosis: Headache vs concussion.
Dr. Martin Bazi-MD. Diagnosis in report: blunt head trauma. Facial contusion
**QUESTIONS/COMMENTS***
1) If the report states that Ava had “blunt head trauma. Facial contusion”, why wouldn’t they do a CT scan? For a patient with a known arachnoid cyst, wouldn’t it be absolutely necessary to make sure the arachnoid cyst was stable after head trauma??
2) WHY was Dr. John Ruge NOT called when we were in the ER on the night of May 5th? I spoke to him earlier in the day. He said that he was going to order a CT scan if we went to the ER. There is no mentioned of the ER doctors calling Dr. Ruge on the night of May 5th.
3) It is stated in the report on May 5th, that there was a “low suspicion for cranial bleed”. If a child with an arachnoid cyst comes into the ER with blunt head trauma and there is any suspicion at all, shouldn’t there be a CT scan ordered?
4) It is listed in the report “To return for worsening symptoms.” We returned on May 8th, 2013.
May 6th, 2013: Ava’s head hurt. She went to bed at 4p and slept until 8a the next morning.
May 7th, 2013: Her head hurt. She slept 5 hours in the afternoon.
May 8th, 2013: I called Dr. Ruge. He wasn’t aware that we went to the ER on Sunday night. We scheduled another MRI for the 10th. However, he had us go into the ER again tonight for a CT scan. We arrived at the ER at Lutheran General at 4:29p. The CT scan was finally ordered during this visit. There was no bleed seen on the CT. It was normal at this time. Her headaches continued to hurt. The pain with the headaches was increasing. We left the ER at 8:12p. Dr Ruge was called during this visit to discuss the CT scan results and Ava’s plan for discharge.
May 10th, 2013: Ava had the MRI at Lutheran General today. She handled the sedation well. She did not drink the silly juice this time. The MRI was stable at this time compared to the MRI on April 8th, 2013.
*Ava continued to complain that her head hurt every single day. She became more melancholy. She played in her room a lot more. She is tired a lot more. She is red (mottled) all over all day. She is either really cold or really hot. She has become more sensitive to noise and bright lights.
May 21st, 2013: Ava’s legs hurt her during the night. She moaned all night.
May 22, 2013: Ava cried from 5a-7a that her legs hurt. She was screaming. I gave her Motrin. She felt better. She got tired in the afternoon. We gave her Motrin again. She went to bed at 3p and slept until 9p. She went back to bed at 10p and slept until 8a the next morning.
May 23rd, 2013: She complained that her head hurt all day. She moaned and cried. She also didn’t complain about her legs.
May 24th, 2013: I called Dr. Ruge today and begged for help with Ava’s headaches. I also mentioned the leg pain she had a few days ago. At one point in the conversation, Dr. Ruge mentioned to me that “maybe we should rescan her. Maybe there is a slow leak from the cyst.”
He also said to push on the Mayo Clinic because he wasn’t sure if the autonomic dysfunction was causing the headaches. He also suggested having Ava meet with Dr. Shah, a neurologist at Lutheran General. He said that Dr. Shah was their “headache expert”. Dr. Shah’s waiting list was out to August. However, Kris, in Dr. Ruge’s office, got us in the following Tuesday, May 28th. We were so relived to get an appointment that soon.
May 25th – May 26th, 2013: Ava complained every day this weekend that her head hurts. She has been taking four to five hour naps in the afternoon all three days. We gave her Motrin Friday night, Saturday night and Sunday afternoon. She said her head is getting worse. She went to a movie on Saturday. We took her to the Arlington Race Track on Sunday. Her head hurt the entire time. She cried almost the entire time today at the race track. She came home and is sleeping again. I gave her Motrin when we got home at 2:30p.
May 27th, 2013: Ava moaned all night long that her head hurt. She started screaming/crying at 5a and didn’t stop until 12p when she fell asleep. It was coming in waves. Her head hurt above her forehead. Both Dr. Ruge and Dr. Shah wanted me to take her to the ER. We went to the ER. They didn’t feel it necessary to scan her again. They just gave her Tylenol. I gave her gabapentin when we got home. She did not eat anything today.
May 28th, 2013: Ava still hasn’t eaten. We went to see Dr. Nisant Shah today. He started Ava on gabapentin today. He explained the following to me:
1) He explained that if the average person hits their head hard, they would have a concussion and be in pain.
2) He went on to explain, if a person that is known to have migraine tendencies and hits their head hard, they would have even more pain with a concussion.
3) Lastly, if a person with an autonomic neuropathy and migraine tendencies hits their head, the pain from a concussion is even more intense then the above two mentioned.
Her head still hurt today after I gave her Tylenol. She complained all day. She did not like taking the gabapentin. It tasted horrible to her. She cried for over an hour because she had to drink the medicine.
May 30, 2013: We took Ava to the ER again at Lutheran General. We arrived today at 3:06p. It is listed on the report that the reason for her visit is “Headache; fever”. I didn’t say that she had a fever. If she had a fever, they never mentioned it to me. Ava rated her pain today in the ER a 8/10. We explained that the headaches come in waves. I mentioned to the ER that Dr. Ruge had mentioned to me on the telephone on May 24th that we might need to rescan her with a MRI. I explained that he mentioned to me that it could be possible that Ava had a “slow” leak that could have started after the May 10th MRI scan. So, Dr. Ruge was called to discuss Ava. It is stated in the report “Discussed with Dr. Ruge – no need for imaging at this time.” It is stated again in the report, “Case was discussed with Dr. Rugi, neurosurgeon on call who agreed with management, no imaging studies at this point.” We left the ER at 5:07p.
QUESTIONS/COMMENTS:
1) Why does it say in the ER report that Ava had “No known head trauma”???? We definitely talked about the fact that Ava hit her head on May 3rd, 2013.
2) It is written in the report “Discussed with Dr. Ruge – no need for imaging at this time.” WHY DID HE NOT WANT TO IMAGE HER AT THIS TIME???? Our question to Dr. Ruge is WHY did he change his mind on rescanning Ava? His gut was perfectly correct. Since her headaches were only increasing in pain and frequency, why did he chose to not listen to his gut? WHY? If Ava would have been rescanned on May 30th, she could have avoided two more weeks of extremely painful headaches and could have avoided how close she came to being blind and dead. Two weeks she endured painful headaches to the point of screaming like a wild animal. Two more weeks. All Dr. Ruge had to have done was order a CT or a MRI on May 30th. However, it is stated in the report “Discussed with Dr. Ruge – no need for imaging at this time.”
3) Progress Note states “Pt discharged to home with mom with no new c/o. Refer to d/c summary.”
4) It is listed again in the report “To return for worsening symptoms”. We returned again on June 11th.
5) Does anyone care that her quality of life suffered because of the pain she was enduring? Her anxiety was at an all time high. Her personality changed from a happy to melancholy child? She did not have one day of fun during all of May and most of June due to her headaches.
June 1st, 2013: Ava cried all day. We drove to Madison to be with family. She was in so much pain that she was unable to even talk to anyone upon arrival. She cried for over an hour about taking her medicine that night. She did not eat again today. She is barely drinking fluids.
June 2nd , 2013: No change with Ava. She is still crying all day that her head hurts in waves. She is not eating. She is barely drinking. We left for the Mayo Clinic today.
June 3rd, 2013: We met with Dr. Ken Mack at the Mayo Clinic as well as Dr. Phil Fischer. Dr. Mack changed her medication from gabapentin to amitriptyline. They put her on IVs for fluids, iron and Ativan this afternoon. She was calm and slept for a few hours while we were in the hospital. The headaches were still there that night. She moaned and cried all night.
June 4, 2013
Happy Birthday to you. Happy Birthday to you. Happy Birthday day dear Ava. Happy Birthday to you.
Ava spent her birthday in a hotel room after being on an IV for hours at the Mayo Clinic.
June 5th, 2013: We took Ava back into see Dr. Mack. She was screaming and crying all morning. She fell asleep by the time we got her into the appointment. No change. We started giving her more Motrin every four to six hours. Dr. Mack did not have anything to add to the appointment. He reiterated that the amitriptyline would take four to six weeks to kick in. We asked about possibly rescanning her at the Mayo Clinic. He did not feel that it was necessary at the time. He was treating Ava for her autonomic dysfunction and not her arachnoid cyst. He was aware that Ava was being seen by Dr. Nisant Shah and Dr. John Ruge at Lutheran General for her arachnoid cyst and posttraumatic headaches.
June 6th & 7th, 2013: No change. She continues not to eat. She barely drinks anything. Her head hurts in waves. She moans and cries all day.
June 8th, 2013: She was up at 4a crying that her head and tummy hurt. She slept most of the afternoon.
June 9th, 2013: She is becoming a shell of herself. She has deep dark circles. She refuses to eat anything. We drove back from Madison today. She fell asleep at 4p until 9p and then again at 9:30p for the night. She moaned and cried all night.
June 10th, 2013: She woke up miserable. She did not get off the beanbag all day. She cried and moaned all day. She refused to eat anything. We got her to drink a little water and other beverages. We had a very high level of concern. Called Pediatrician to see what the warning signs are for dehydration. They said as long as she isn’t vomiting or has diarrhea, then she is still hydrated. She has absolutely no energy and her tummy hurt all day too. She had Motrin at 8a and 3p today and again at 9p.
June 11th, 2013: Ava was up all night. She woke me up around 12:30a crying that her head hurt. She moaned and cried all night. I could not get her back to sleep. I gave her Tylenol at 3:30a. Her tummy started to hurt too. I got her to eat a few bites of cereal and toast at 5a. She finally fell asleep at 5:30a. She sat on her pink beanbag all day long. She didn’t eat. She didn’t walk. She screamed and moaned all day. We took her to the ER again at Lutheran General. We arrived there at 1:49p. The report lists Ava’s Visit Reason as “Headache; ha” It is listed in the report that Ava’s headache was a 10/10. She told them that she was nauseous earlier in the day. They put her on one round of Toradol, NS bolus and Benadryl. Since it didn’t make a difference, they encouraged us to take her home. This was the day that the doctor said, “Dr. Shah does not feel it necessary to do any more testing.” They sent us home on Tylenol. She continued to have 10/10 pain all night. She screamed and screamed all night long.
In the report, it states that Dr. Singh spoke to Dr. Shah. Per Dr. Shah, the ER gave us the following three options:
1) DC Home. Increase amitriptyline from 10mg daily to 10mg BID
2) DC home. Remain on current dose of amitriptyline
3) Admit to general pediatric floor for IV pain control on Toradol.
It states in the report, “If family decides to go home, they are to call Dr. Shah’s office in 1-2 days with a progress report. He will determine FU at that time.”
QUESTIONS/COMMENTS:
1) If we would have followed Dr. Shah’s advice about waiting 1-2 days to call him, what would he have done? Her level of pain was a 10/10 on June 11th. Waiting two more days was ok for him??
2) The ER doctors and Dr. Shah***NEVER MENTIONED SCANNING HER AGAIN***
3) The ER doctors and Dr. Shah***DID NOT TAKE A NEW CT SCAN IN THE ER***
4) Dr. Shah ***DID NOT MENTION ORDERING A NEW MRI IF WE ADMITTED HER***
5) The ER doctor***encouraged us to go home since the first round of pain medication did not break the cycle***
Progress Note from Kerry Lynn Murphy: “CCLS followed up with pt and family. Pt lying in bed tearful asking for her IV to come out. CCLS provided emotional support to pt and parents. Parents expressed frustration about not having answers about pt’s pain. CCLS encouraged them to take one step at a time. Pt is waiting to be discharged home and has no other needs at this time.”
Again, how much pain is ok for a 6 year old? How many trips to the ER did we need to do to save her sight and her life?
Again, Ava did not have one day of fun in May or June due to her headaches that were now a 10/10 all day and all night.
June 12th, 2013: We brought her new puppy home. She had no interest in him. She spent the afternoon in our bed. She slept with us. She was miserable and screamed all day and all night.
June 13th, 2013: We took her to the ER at Children’s Hospital of Wisconsin. We were hoping to have her admitted and scanned. They brought down their neuro doc. He wanted us to come back another day for a consult. We looked at him and said, “We are not leaving.” The ER doc agreed. He could not send us home. He said she was in too much pain. He admitted us for the night. Ava screamed like a wild animal all night long. They gave her the following pain medicine…in this order:
Toradol
Compazine
Benadryl
Valproic Acid
Valium
Valproic Acid
**None of the above took her pain away. At 4:30a, they gave her Morphine. She finally fell asleep for two hours
June 14th, 2013: Ava was scanned at the Children’s Hospital of Wisconsin today. She had the following scans: MRI, MRA, MRV. She also had a lumbar puncture (spinal tap) today. Her pressure was at 55. The normal pressure range is 20. They were able to drain the fluid for testing as well as to reduce her pressure to 27. They couldn’t take more fluid out at the time. They would have been putting her in danger. Her headaches improved immediately. Unfortunately, her vision did not and that is why we had the procedure done immediately to save the vision.
June 15, 2013
Ava had brain surgery today at Children’s Hospital of Wisconsin. Ava’s arachnoid cyst ruptured and there were new bilateral subdural fluid collections. The lateral subdural hygroma was causing severe papilledema, which could have caused blindness. They suggested operating on her immediately for the ruptured arachnoid cyst as well as the intracranial pressure. Ava’s sixth cranial nerve was being squeezed by the increased intracranial pressure, which gave her weakness in her right eye. She had double vision from the papilledema. She will still have the double vision for a while. Unfortunately, this will take several weeks to improve. The good news is that the pediatric ophthalmologist came by for the post-op follow-up. Her vision is back to 20/20 when it had been 20/60 and 20/40 previously because of the pressure. However, her double vision will still take time to get better.
She also had a lumbar puncture (spinal tap) yesterday. Her pressure was at 55. The normal pressure range is 20. They were able to drain the fluid for testing as well as to reduce her pressure to 27. They couldn’t take more fluid out at the time. They would have been putting her in danger. Her headaches improved immediately. Unfortunately, her vision did not and that is why we had the procedure done immediately to save the vision.
Ava is recovering in the ICU today. We have a MRI tomorrow to check for swelling, pressure, bleeding, etc. This will determine whether she will have any more surgeries immediately and/or how long we will stay here before being released; a lot of it depends on how well Ava responds post-op. There is a significant chance that Ava may need multiple surgeries in the future. They drained the subdural hygroma via burr holes rather than fenestration of the arachnoid cyst. She has a drain coming out of her head.
There was also a situation going on behind the scenes. They had given Ava two different medicines during the day that could potentially make her bleed more heavily during surgery. They had to call in the hemoglobin specialists to go over the risks of the surgery since she was given the two medicines. They “typed” her blood during the night before surgery. They needed to have extra blood available during the surgery if she started to bleed out. Fortunately, it wasn’t an issue.
If you have time to read the following, here is Ava’s story….
Ava had been having severe headaches since May 3rd. We were not sure if it was from hitting her head on a coffee table while playing with her babysitter or due to her autonomic neuropathy and the warmer weather.
I called her neurosurgeon on May 4th & May 5th. I was concerned about her headaches after hitting her head. We took her to the ER at Lutheran General on the evening of May 5th. They sent us home without even doing a CT scan. I called her neurosurgeon again on May 6th. I told him that they did not scan her in the ER the night before. He said to schedule a MRI as soon as possible. Unfortunately, we couldn’t get the MRI until the 10th. She was still in so much pain that I took her back to the ER at Lutheran General on May 8th. They gave her a CT scan that night and it was normal. They sent us home with Tylenol. We had her MRI done on the 10th. Her arachnoid cyst was stable. However, her headaches continued to persist. They gradually became more and more intense. I took her to the ER at Lutheran General again on May 17th. They sent us home.
On May 24th, I called Dr. Ruge and begged for help. He put us in touch with a new neurologist, Dr. Shah. His waiting list was through September. Dr. Ruge’s office got us in on May 28th. Dr. Shah met with us and put Ava on gabapentin.
I also reached out to Dr. Phil Fischer at the Mayo Clinic. He put us in touch with Dr. Kenneth Mack, neurologist. He had a waiting list through August. However, Dr. Fischer’s office got us in on June 3rd. We met with Dr. Mack. He put Ava on IV fluids, iron and Ativan that afternoon. Her headaches only improved for the evening. He took her off of gabapentin and started her on amitriptyline.
On June 4th, Ava celebrated her 6th birthday in pain. She did not even want to buy a birthday cake or open presents. We still have several presents sitting on our dining room table left unopened.
On June 5th, Ava was screaming all morning. We called Dr. Fischer and Dr. Mack at Mayo and asked for help. Dr. Mack met with Ava again. He didn’t have any suggestions. He felt that the amitriptyline needed time to kick in. Dr. Fischer called us on the evening of the 6th. We told him that Ava was screaming again all day and all night. His suggestion was to max her out on Motrin for five days as well as to continue the amitriptyline.
June 11th, 2013, Ava was up all night. She woke me up around 12:30a crying that her head hurt. She moaned and cried all night. I could not get her back to sleep. I gave her Tylenol at 3:30a. Her tummy started to hurt too. I got her to eat a few bites of cereal and toast at 5a. She finally fell asleep at 5:30a. She sat on her pink beanbag all day long. She didn’t eat. She didn’t walk. She screamed and moaned all day. We took her to the ER again at Lutheran General. We arrived there at 1:49p. The report lists Ava’s Visit Reason as “Headache; ha” It is listed in the report that Ava’s headache was a 10/10. She told them that she was nauseous earlier in the day. They put her on one round of Toradol, NS bolus and Benadryl. Since it didn’t make a difference, they encouraged us to take her home. This was the day that the doctor said, “Dr. Shah does not feel it necessary to do any more testing.” They sent us home on Tylenol. She continued to have 10/10 pain all night. She screamed and screamed all night long.
On June 12th, Dr. Fischer, our Autonomic Specialist at Mayo, wrote me the following email:
“I don’t have any magic to suggest beyond the 20 mg per day of amitriptyline and the oral fluids and the emergency department if/as needed. Unfortunately, it sounds like Ava has not pulled out of the vicious cycle of inactivity-poor sleep-poor intake-pain. Whatever “little” steps you can take toward normalcy along the way (even a gentle walk outdoors) will likely help re-set the neurotransmitter balance a bit.”
She became lethargic after we came back from Mayo. She stopped eating. She barely drank any fluids. Her pain was an excruciating 10…red on the chart of faces. She screamed all night and all day. It was the most heartbreaking experience of our lives….
One June 13th, we took her to the ER at Children’s Hospital of Wisconsin. We were hoping to have her admitted and scanned. They brought down their neuro doc. He wanted us to come back another day for a consult. We looked at him and said, “We are not leaving.” The ER doc agreed. He could not send us home. He said she was in too much pain. He admitted us for the night. Ava screamed like a wild animal all night long. They gave her the following pain medicine…in this order:
Toradol
Compazine
Benadryl
Valproic Acid
Valium
Valproic Acid
**None of the above took her pain away.
At 4:30a, they gave her Morphine. She finally fell asleep for two hours
A few weeks earlier, in my search for answers with Ava, I had her evaluated by the Genetic Clinic at the Children’s Hospital of Wisconsin. We met with Dr. Gunter Sharer, a geneticist, and Linda Carey, a genetic counselor. We discussed having Ava and the other kids evaluated by the Autonomic Clinic at Children’s Hospital of Wisconsin in the next few weeks. I had called them several times to see if they could get us in sooner than later because of Ava’s head. Unfortunately, I didn’t hear back right away.
On June 14th, we decided that we were going to take her somewhere. We really, really did not want to go back to Lutheran General. Fortunately, Linda Carey called me back. I pleaded my case for Ava to get into their hospital. She said that it takes awhile to get into the clinic. I said that I was desperate to get help for Ava. I told her that I was taking her to their ER. She said she would do what she could to get Ava into someone in neurology.
We left for Milwaukee right away. The ER was not sure how to handle Ava. They didn’t have an answer for her pain. Neurology came down. They said it would be better if we came back for an evaluation in the morning. Justin and I looked at each other. We told them that we were not leaving. The ER doctor that came back in agreed. Ava was definitely in pain. He was going to admit her immediately.
So, the long…painful journey for Ava isn’t over yet. She is still in a lot of pain. However, we do have answers and we saved her sight and mostly likely her life. Thank you to the doctors here in Milwaukee.
Thank you ALL for your continued support, love and prayers.
Love,
Gina
June 25, 2013
Ava is having brain surgery again tomorrow. She was having headaches over the weekend. She woke up on Monday morning with a 10/10 headache as well as double vision. We brought her to the Children’s Hospital of Wisconsin immediately. They admitted her last night. They gave her a quick sequence MRI to check if there were any changes. Unfortunately, she still has fluid in the subdural space around her brain. It is mostly over the frontal lobes. The fluid is greater on the left side where her arachnoid cyst is located. We assume that the arachnoid cyst is leaking. We have no other cause to suspect at this time.
They will put a drain back in her brain. Fortunately, they will use the same burr holes from her previous surgery. She will keep the drain in her head after the surgery. They told us to expect to stay at least a week this time. They want her to drain for a longer period of time. She calls the drain out of her head her “string.”
She is aware of the surgery tomorrow. She is really scared. She does feel comfortable being back in “her room” here at the hospital. We are in the same room as we were during our last visit.
Thank you for all of your wonderful emails, texts and phone calls. Please know that we feel truly blessed to have so many people praying for Ava as well as our entire family.
June 28, 2013
Thank you all for your wonderful emails, texts and phone calls. We truly appreciate all of your prayers, love and support.
Ava is stable after surgery. She still has the drain in her head. She is draining around 350cc per day. She is apparently not reabsorbing enough of her CSF (Cerebral Spinal Fluid), something that happens normally in the brain. This was what caused the buildup of pressure in her head again and required the reinsertion of the drain.
They plan to have another MRI either tomorrow or Sunday to see the status of the fluid inside of her brain. She will continue to drain over the weekend and into early next week. They will eventually clamp the drain and see how she does on her own. We will push for clamping her for at least two or three days before they take the drain out. We will want to make sure that she is reabsorbing the fluid herself before we take her home again.
If she continues to have difficulty with pooling of the CSF and intracranial pressure, we will have to do what is called a fenestration of the arachnoid cyst. This would be a major surgery with the possibility of a future subdural peritoneum shunt catheter. We will keep praying that she will start to reabsorb more of her CSF on her own.
Ava is in good spirits. However, she is not eating or drinking very much. We are working on it with the help of the nurses. They get to play the “bad cop” once in a while, which gives me a break. She was very weak but is now getting stronger. We took her out for several wheelchair outings today. It definitely helped lift her spirit. She sings and plays with her toys in her bed. The nurses love to listen to her sing.
We appreciate all of our visitors. It means a lot to us. However, please know that she is aware of all the people that are thinking about her from far away. I love to tell her when I get a text or an email from someone asking about her. It always makes her smile.
Thank you again for loving and praying for our Ava.
Love,
Gina
July 1, 2013
Ava had another MRI this morning. There is no change in the scan. She still has a lot of fluid in her brain on the left side. She is still putting out an average of 350cc daily. The CSF looks clearer than it did when we first started the drain last Wednesday. Hopefully, it will make a difference with reabsorption. The doctor is planning to take out the drain or clamp the drain tomorrow. The drain has been in too long and there is a risk of infection. Most likely, they will take it out. They want to see how she does on her own. They had moved the drain around to different levels of pressure. Today, the drain was set at 10. She started to leak fluid out of the top of her head. They lowered it to 5.
As in our previous email, the next step is fenestration and/or a shunt. They will still observe her for a few days here in the hospital. We may be able to take her home over the weekend. The doctor is confident that we are aware of what the warning signs would be if the pressure started to build up in her head again. Also, the doctor will be out of town from July 4th until July 8th. So, we need to hold off on surgery for two reasons. We need to see if Ava can reabsorb the CFS on her own and give her body time to do it. And, wait for the doctor to get back in town!
Love,
Gina
July 2, 2013
Ava had her MRI yesterday (7-01-13). There was no change in the scan. She still has fluid in the subdural space around her brain. It is mostly over the frontal lobes. The fluid is greater on the left side where her arachnoid cyst is located (This is the same as the MRI on 6-29-13).
Dr. Foy, her neurosurgeon, stopped by this morning. He decided to take out her drain today. There is a risk of infection with leaving it in and/or putting in a new one. They plan to keep her here for observation for at least 24 hours. Dr. Foy is out of town as of tomorrow and back on Monday, June 8th.
They gave her morphine and then took out the three staples and the drain. They pulled her strings (stitches) again to close the hole, which was extremely painful. They also put two staples over the incision. Their hope is that she will not leak the fluid out of her incision. We are nervous. She was still draining over 300cc of fluid as of yesterday. She drained all night as well.
They will watch the incision to see if she leaks anything. She leaked out of the incision yesterday. The drain actually twisted like a garden hose. They had to use Popsicle sticks to straighten it out.
In theory, the drain was closed yesterday due to the drain being twisted. When it was twisted, she leaked out of the incision. Therefore, her brain wasn’t holding the amount of CSF in yesterday. What is going to change today, tomorrow or the next day? When I asked the doctor, he said they just hope it starts reabsorbing. Every child is different. He said that they have had two “failed” drain attempts. The next step is fenestration of her arachnoid cyst. This would be a major surgery with the possibility of a future subdural peritoneal shunt catheter. To be very honest, both of the next steps are very scary to us and make our hearts hurt for her.
Dr. Foy stated today that Ava has proven to have some time before she gets into danger. She went somewhere around three to five weeks with the buildup of intracranial pressure the first time around before her eyes were in complete danger of blindness. The second time around with the drain, she had gone about 6 days from when the first drain was taken out. This morning, he felt we should be ok to go home over the holiday weekend. He thought it would be good for Ava as well as for our family to get out of the hospital for a few days. If we run into any problems over the weekend, we are to bring her back and work with one of his partners.
Well, I wrote this email update around 2p today. Since then, Ava’s headaches have returned. She is at about a 6 (pain level) right now. She is already putting her hand over her eyes when she watches her DVD player. They paged neurologist on call. They said to “watch her” over the next few hours. Our journey continues…
Please pray that she doesn’t leak through her incision. Please pray that her body starts to reabsorb her CSF. Please pray that her headaches stop and that there is no intracranial pressure building again. Please pray that her eyes remain safe.
Again, thank you for your emails, texts and phone calls.
July 3, 2013
We had an emergency CT scan last night. It showed more fluid building up, which is creating more intracranial pressure. The CT also showed more left to right midline shift of her brain.
Because her symptoms returned so quickly and the result of the CT, Ava is scheduled for her 3rd brain surgery later today, probably around 5p. They are going to do a craniotomy and fenestration of the cyst into the cerebrospinal fluid spaces. It will not be done endoscopically. We have a different doctor doing this surgery, Dr. Foy’s partner, Dr. Lew. He just walked us through the surgical procedure. The surgery will take around 3 hours.
They are going to do a craniotomy where they will open up a half-dollar size opening in her cranium; they will then create a pathway through the arachnoid cyst that will hopefully drain her CSF into the basal cistern. In addition, we will put the drain back in for the first 48 hours to help reduce the pressure immediately and give the pathway a chance to work. The success rate of this procedure is somewhere above 70%. The success rate is related to not only creating the proper pathway but also if Ava’s body/brain can absorb the CSF on a cellular level. The procedure gives the CSF a bigger space to drain and immediately should reduce the intracranial pressure. We should be able to see an improvement/success within the first 48-72 hours. If we don’t see improvement, then we would go immediately to the shunt procedure. The shunt procedure has a success rate of 100 pct but there are long term complications and the shunt is a permanent apparatus that we would like to avoid if at all possible.
The risks and complications associated with this surgery really relate to how close the doctors will be to the carotid artery and optic nerve. So there is a possibility of eye damage, bleeding, etc. Doing a craniotomy rather than using an endoscopic procedure allows the surgeon to use both hands and work around the optic nerve and carotid artery. It is a conservative approach that allows for more control to give the best chances of avoiding these possible complications. After the surgery, Ava will have some bruising and swelling around her left eye because of the temporal muscle being a part of the surgery.
If everything goes well we could be out of here by Sunday/Monday. If not, we will be here about 10-12 more days if we have to do the shunt procedure.
Ava is aware of her surgery later today. She is scared. We are trying to keep her calm. She is very disappointed that she is not going to Grandma and G-Daddy’s house today to see Oskar and Lucy. She was also looking forward to the fireworks tomorrow. She will be in ICU tonight. Hopefully, we will return to “our room” tomorrow afternoon. The nurse said there is a good view of fireworks from this room.
Thank you again for all of your emails, texts and phone calls. Please pray for our little girl today.
July 4, 2013
Thank you so much for your email. It always means a lot to hear from you.
Surgery went well last night. The doctor thought it was a success. However, we won’t know if it actually worked for at least 48 to 72 hours. She does have double vision again. The doctor said that is to be expected at this time because of the swelling around her left eye. She also has some numbness in her left cheek. I hope both of these get better soon. She came out of sedation very angry. We eventually got her to sleep with a lot of morphine.
She continues to drain a lot today. Unfortunately, draining isn’t a good sign. The fluid is supposed to start draining into the CSF passageways. She also still has the headaches. They are still coming in waves. This is also not a good sign. The drain will stay in for a few days and then they will clamp it. We have to pray that the headaches are gone then. Realistically, I’m preparing myself for the shunt.
It’s been a really hard day. The scar is horrible on the side of her head. It’s between her ear and her hairline/face. It’s about five inches. I wasn’t expecting it to be so large. The drain comes out of it. She is still miserable. Sad. Depressed. Today has been one of the hardest days. I’m not sure if it’s because it’s the Fourth of July or because she is so miserable.
Justin left for Madison to see Oskar and Lucy for the night. I figured it would be better for him to get out of here and see the kids. We are hoping to see the fireworks from our room.
I pray the fenestration works and we won’t need a shunt. I pray to get out of here sooner than later. I pray that Ava will be healthy soon. I pray to be back together as a family soon. I pray, I pray and I pray.
Love you,
Gina
July 5, 2013:
Ava did not see the fireworks last night. She was asleep. Her head was hurting a lot last night so they gave her morphine to calm her down. I watched the fireworks from our room by myself. There were about 15 different locations for fireworks out our window. I would have loved to have watched them with her. I played Canon in D the entire time.
Today has been another very difficult day. Ava woke up with her left eye swollen shut and double vision in her right eye. She is extremely sad. Her headaches have come and gone a few times today. Unfortunately, they did reach an 8/10 a couple of times. She wants the bed to be flat. It’s the only position that makes her feel comfortable. However, the doctor told us she needs to sit up today. Her head hurts when she sits up or stands up. The nurse was pretty tough with her today. It was hard for me to handle. She made her go to the bathroom. Ava screamed and cried the entire time. She was dizzy and couldn’t keep her balance. Her head was hurting as well. The sound of the nurse and I talking was hurting her head. The nurse is also threatening a food tube up her nose if she doesn’t start to eat and drink. She told her she has until Sunday.
When the doctor came in this morning, he said he didn’t want to scan her yet today. He thinks he might scan her tomorrow. As long as she is draining, he doesn’t think it is necessary to scan her. Well, she is definitely still draining. They dumped the bag of CSF this afternoon. It had been filling up ever since the surgery. The CSF was over 600cc when they threw it out. It is still red/yellow. It is not even close to being clear.
As of last night, I was positive she was going to need a shunt very soon. However, the doctor said today that he still isn’t “sold” on it. He wants to give her more time to prove herself. As a parent, waiting for her to “prove” herself is heartbreaking and very, very difficult. We all pray that Ava won’t be “shunt dependent” the rest of her life. However, this journey must have a happy ending. If a shunt takes her pain away, sign me up.
Love you,
Gina
July 6, 2013:
Last night:
Ava fell asleep at 4p yesterday. I sat and watched her sleep as I worked on her journal and read a book. I asked the nurse at 6p what the plan was with her schedule. She said that she was going to wake her up at 8p to check her vitals.
At 6:30p, Ava woke up screaming that her head hurt. I told the nurse. She didn’t comment on it at all. Ava was miserable. Chrissy got here at around 7p. She came to sit with her while Justin and I went out to grab dinner. As we were walking out of her room, we ran into Ava’s nurse as well as the head nurse, Michelle. Michelle asked how Ava was doing. I said that she had a really bad headache right now. I asked if we should stay. They encouraged us to go.
Around 7:30p, Chrissy texted that the neurology fellow was in the room flushing the tube/drain. I texted her back, “What is happening??”
Ava has been draining a lot of blood with her CSF since she had the surgery. At the time last night, there was a blood clot that clogged the tube/drain. The possibility of blood clots in her drain was never explained to us by the doctors or by the nurses. Ava didn’t drain from 4p-7p yesterday due to a blood clot in the drain. The intracranial pressure began to build again in her head, which caused her headache. Thankfully, the head nurse pushed the younger nurse for answers. Michelle is the one that paged the neurology fellow last night. Not Ava’s assigned nurse.
Upon my return from dinner, I questioned the nursing staff again as to why, as parents, we are not included in the process with our own child? Ava stopped draining for three hours. How hard would it have been to say, “Hooray! Ava stopped draining! The fenestration must be working!” or “On no! Ava stopped draining! There must be something wrong with the drain!” Instead…..we got nothing from the nurse. They promised that they would communicate better with us in the future.
A new nursing shift started around 11:30p. We had Crystal. She was wonderful. However, Ava and I didn’t sleep at all during the night. Unfortunately, Ava’s head hurt the entire night. She was given morphine several times. I usually sleep in Ava’s bed at night. She needs the physical contact. Last night was the first time she asked me to keep my hand over her heart. She said, “I like to cover my heart when I’m scared or in pain. It makes me feel better.” I kept my hand there all night long.
We woke up late to find the neurosurgeon in our room. He explained that Ava will have another MRI today. He wasn’t sure at what time. He said she is still continuing on the path of uncertainty. He explained that as long as there was blood draining with the CSF, there is no way to know if the fenestration worked. We have to continue to wait until her CSF is clear. At 5p today, her CSF is still blood red. How many more days will she drain CSF with the blood? No one knows. Every child is different. UGH!
Once we get to the day when Ava stops draining blood in her CSF, they will clamp her drain for 48 to 72 hours to see if she starts reabsorbing the CFS on her own.
She’s only been out of her bed twice today. Both times, it was very painful for her to be upright and walk. She can’t see very well and loses her balance. Ava is still very angry. She is still very depressed. She is still very scared. She still has 8/10 headaches in waves.
She is also still not eating or drinking very much. They are forcing her to drink Pedialyte Shakes and chocolate milk with Miralax. They told us to prepare ourselves for tomorrow. If she doesn’t improve tonight, they plan on putting feeding tube up her nose tomorrow. Are you serious?? I’ve already threatened to barricade her door. I’m not sure I’m going to be able to handle it. Justin and I have explained to her over and over the importance of eating and drinking today. Unfortunately, she is not listening.
So, please pray that Ava does not need a feeding tube tomorrow and that the blood in her CSF stops soon.
I would also like anyone and everyone to please send good vibes my way… I need all the help I can get that I will be calm tomorrow and that security does not remove me from the hospital if the doctors do decide to put a feeding tube up my daughter’s nose.
Love,
Gina
PS: At 6:30p, Ava’s headaches reached a 10/10. We paged the neurology fellow just to touch base. He came right away. He noticed that Ava’s drain wasn’t draining. He was going to flush it. I asked him why we do not just flush it twice a day as a precaution. He said that the risk for infection is higher every time you flush it. As he was about to flush the drain tonight, he noticed it was clamped shut. One of the nurses, the same one that saved Ava last night, Michelle, left the drain closed. It was closed for about 45 minutes and caused Ava to have a 10/10 headache. She came right in and apologized. I told her it’s fine. He didn’t actually flush it, which means he didn’t increase her chance of infection.
PPS: At 7p, we got Ava up for her 3rd time today to go to the bathroom. Both Justin and I dread it every time. She is just awful to us. She is just awful to the nurses. As we were putting Ava back in the bed, I started to cry tonight. The nurse looked at me and asked if I needed a moment to step out. I shook my head no. She looked at me and said, “This is horrible for everyone.”
July 7, 2013:
Last night:
The last two days were pretty miserable. However, I felt that the night ended with a message from above…
Although Ava didn’t want to eat anything yesterday, she wanted to order a pizza. I went downstairs to wait for the delivery guy. I sat down on a bench in front of the hospital. As I sat there enjoying the fresh air, an older couple and a nurse came out of the hospital. They were very upset. The wife went to get the car. The nurse sat down with the older gentleman. I overheard their conversation. Their grandson died an hour before. His son and daughter-in-law were still upstairs holding their four-week-old in their arms. He arrived at the hospital brain dead. They had to let him go. They didn’t have any answers as to why he was dead. They would have to do an autopsy. I just started bawling in front of them. I turned to the older man and the nurse and gave them my deepest sympathies. I briefly explained to them that my daughter was upstairs after three brain surgeries. My story just didn’t seem as important. I gave the older man a hug before he got in the car with his wife. He thanked me and got in the car. The nurse walked me back up to my floor. I asked her if she walks out a lot of families after a death. She replied, “It happens more than I like to admit.”
Here I had been sitting in my room all night as I watched Ava starve herself. I was feeling sorry for MYSELF. I was complaining how tired I was of sitting in this room. I was complaining how I just wanted this journey to be over. I was complaining that I could not do this one more day. WOW. I didn’t mean it at all. I didn’t mean it at all. I didn’t mean it at all.
I felt the message from above was the following….Keep fighting for Ava. Keep your head up. Keep calm. Keep rubbing her back. Keep her strong. Breathe. Don’t ever give up. Ava needs me. I need Ava. She’s still alive and she is going to make it through this. We are not going to get walked out of this hospital by a nurse. I am not leaving here without my Ava.
I went back upstairs and got into Ava’s bed. I held my hand on her heart until we both fell asleep. Unfortunately, we didn’t sleep very long. Ava’s headaches returned in full force. I recorded them so the doctor could hear them in the morning. We had a new nurse. She was Jamaican. She was beautiful and calm. She told us, “Ava, you need to be calm in order for your body to heal.” Again, I cried. No one had ever said that to me before. It makes sense. She gave Ava morphine. We fell asleep.
We woke up almost every hour on the hour. Ava’s head still hurt. She would scream for about three minutes and then fall back asleep. It’s a ritual that I am very familiar with. This is what she has done now for over two months. Why isn’t she getting better? The calm, beautiful nurse gave her more morphine at 4a. We feel back asleep.
At 5a, Ava woke up screaming and itching all over. I turned the flashlight on my iPhone on. I looked at her body. She was covered in huge red welts. She was itching like crazy. I paged the nurse. She explained that it was possible that the morphine caused Ava to have a histamine release. She gave her Benadryl. I scratched her body until we finally fell asleep at 6a.
At 6:30a, the neurology fellow came in. He checked her eyes and her drain. I told him that we were up all night with really bad headaches and a terrible rash. He said, “She looks better now. She seems fine.” I offered to play my recording to him. He said that it was not necessary. I was half asleep so I agreed. As he walked out of the room, I asked him to please tell everyone to let Ava sleep in this morning. Please do not do the feeding tube at 10a. He agreed and walked out.
Ava and I slept until 9a. When I walked out to talk to the nurses about Ava’s feeding tube, they explained to me that the nurse from the night before fought my battle for me. She put in the records that Ava need to have a psychologist, a nutritionist and a child life specialist there in order to do the feeding tube. Since it was a Sunday, the nurses were unable to get all of the specialists there. Ava was granted one more day to prove herself.
When the Head of the Neurology department came in at 10:30a, he agreed that Ava didn’t need the feeding tube today. However, he did want to take an X-Ray of her abdomen. He felt that she was probably full of you know what and it made her not feel like eating. He also explained that there was really no change in her CSF. We were to stay the course again today. We were to wait for the CSF to clear all of the blood. We were also to get Ava to poop and to eat today. The plan was set for the day with the nurses. My only question….Who was the one that ordered the feeding tube? The nurses? I guess that they have a certain protocol for what a child needs to eat/drink and Ava wasn’t meeting their protocol. What would have happened if I hadn’t pushed them to give her another day? I knew in my heart that Ava didn’t need a feeding tube today.
Justin spent the entire day getting Ava to drink her Pedialyte and her Gatorade. He did a really great job with her. He set a timer for every single minute over three hours. She had to take a drink when the bell rang. It worked.
Ava continued to drain blood in her CSF all day today. Her headaches were a little better today. She wasn’t given any morphine. The neurosurgeon was right. Ava did need a suppository. She got one around 5p. She did “the job” around 7:30p.
At 7:45p, the headaches began again. She is getting morphine injected into her IV as I finish typing this entry.
We are still trying to get her to eat something before we call it a night. The feeding tube debate will continue in the morning.
Love,
Gina
July 8th, 2013
Last night:
Ava was given her first double shot of morphine last night as well as Benadryl to knock her out at around 7:45p/8p after she was having one of her painful headache episodes. I crawled into bed with her around 10p. I woke up to her screaming again at 12a. The nurse gave her morphine again. It took us about an hour and we fell asleep again. I woke up to her screaming at 3a. The nurse gave her morphine again. It again took us about an hour to fall back asleep. I woke up to her screaming at 5:45a. The nurse said that the neurology fellow was out in the hall. For the first time, he actually was able to witness one of Ava’s episodes. He said he was going to order another MRI and told the nurse to give her more morphine right away. For the first time, I saw the hurt for Ava in his eyes. He said, “I’m sorry she is still in pain.”
This morning:
We have a new nurse today. Her name is Amy. She’s fine. She sounds positive towards Ava as we try to get her up and ready for the day. I asked about the feeding tube. She said that there isn’t anything in the notes about it this morning. Ava asked if we could go downstairs to the gift shop to spend the $20 that Grandma gave her. I told her we would do it after we got her ready. The nurse agreed to the reward. After Ava was dressed and holding her $20, the nurse got a page. She then told Ava, “Sorry. Can’t do it.” Ava was upset and disappointed. She voiced her disappointment to the nurse. The nurse just walked out.
I went out to the nurses’ station and politely asked, “If there is a chance any of the nurses could take Ava for a walk, we would really appreciate it.”
The nurse finally came back and took us for a walk. Her tone with Ava was rude and unprofessional. She rolled her eyes several times. Ava wanted to buy something herself. She was given the money. The nurse made a comment about how many things she already had in her room. I just about lost it. However, I didn’t say anything. I helped Ava spend the money on a dinosaur kit. It is a block of sand with dinosaur bones in it. She spent most of the day playing with it in her bed. Every single person that came in the room today was fascinated by it. It was priceless.
I met with a nutritionist today. She brought a lot of information for us to read about what foods/drinks provided Ava with good protein, etc. She helped us find a drink that was different than the Pedialyte shakes. Ava seems to like the Ensure Clear. The nurses agreed to it.
Around 10a, Dr. Foy came in. He is Ava’s original neurosurgeon. He’s back from vacation. Ironically, his time off was spent in Barrington, IL. He saw the fireworks and the parade that Ava would seen if we were at home. His parents live in Barrington. He went to the Barrington High School. He graduated high school in 1995. He’s younger than I am. Anyway, we really like him. It was good to see him again. Until…he told us that we were going to do the fourth surgery. I knew it in my heart already but I didn’t really want to hear it. He walked me through the reasons why we need to do the shunt.
Dr. Foy did not order a MRI today. He already knew what it would show us. No change. As previously discussed, Ava is not reabsorbing the CSF. She is still draining way too much fluid. She is still having headaches from the intracranial pressure. There is still fluid in her subdural space. It’s obvious to Dr. Foy. The fenestration did not work.
He told me that we would do the surgery at some point this week. I didn’t really hear why we were waiting. I’m not sure if it is because Ava still has blood in her CSF or because there isn’t room on the surgery schedule. I’m hoping to find out in the morning. All he said was that Ava would have surgery at some point this week. I was trying to listen to him and focus on the conversation. He was talking to me but the words were going over me. I was listening but I couldn’t hear him. It was really weird moment. It was like everything was moving in slow motion. The nurse kept interrupting him. I finally turned to her and said, “Please. This is my time with the neurosurgeon. I’ve waited a long time to talk to Dr. Foy. You just met us a few hours ago. Please let us have a conversation directly.” She took a step back. I did not apologize.
I asked Dr. Foy to walk me through the upcoming surgery. I asked if the arachnoid cystoperitoneal shunt would go through the same hole they used for her fenestration. I remember that Dr. Lew said that Ava’s skull was really soft right there. He explained that the shunt, most likely, would be put through the original burr hole on the top of her head. There would be a total of three incisions. There would be another one in the back of her neck/skull and the last one in her stomach. The cysto-peritoneal shunt drains to the peritoneal cavity. Again, I think this is what he said. I definitely need to hear it all again. Justin will need to be informed as well. It’s a lot to process.
I met with the psychologist today after Dr. Foy. She spent an hour with me discussing Ava. She informed me that the nurses felt that it was hard to do their job with Ava because she was difficult. I was calm. I did not get upset. I like the psychologist. Her name is Nina. I wanted to work with her for Ava’s sake. It was exhausting. I fought for Ava’s respect. I have felt that some of the nurses think being rude back to Ava will work. Well, Ava is six-years-old. Being rude back to a six-year-old seems ridiculous to me. I asked that they talk firm but respectful to her. It was a long negotiation. I had to agree to a sticker chart. It’s fine. She gets a sticker for going potty without complaining. She gets a sticker for saying thank you. She gets a sticker for eating a piece of food. It’s fine. She likes picking out the stickers for the chart. I don’t think it fixes the problem. She’s been cut open three times. She has a hole in the side of her head. She has a drain coming out of her head. She has a central line sticking out of her arm. She’s angry. I’m sorry she isn’t nice all of the time. I don’t think I would be either. And, the fact is that she is going to be cut open again in a few days. Give her a break! Ok. Sorry. Needed to vent.
I did work with the nurses the rest of the day. I was nice to all of them.
At 7p tonight, there is still blood in the CSF. I’m confused as to how this is ever going to change. Hopefully, I will get more answers tomorrow.
July 9, 2013
Thank you again for all of your phone calls, emails and texts.
I thought it was time to update you again on Ava’s progress. We are still here at the Children’s Hospital of Wisconsin. We all know that Ava underwent a craniotomy fenestration of her arachnoid cyst on July 3rd, 2013.
At the time, Ava’s surgery went well. The three major risks during surgery were stroke, blindness and death. Thankfully, our prayers were answered by all of you. Ava conquered all three of them.
The neurosurgeon that performed the surgery, Dr. Lew, told us that the success rate for a fenestration of an arachnoid cyst was between 70%-80%. Unfortunately, Ava is not going to fall into the success rate percentage. She is still producing over 300cc of CSF daily. She is still having headaches on a 10/10 scale. She has not improved after six days of drainage. Actually, we were supposed to be going home today.
The next step in this journey is to clamp her drain tomorrow to see if she reabsorbs her CSF on her own. During the last few days, Ava has been clamped around 45 minutes a day. Unfortunately, she has intracranial pressure built in her head immediately after every time she is clamped.
The next step after the failure of the fenestration is for Ava to have a permanent cysto-peritoneal shunt. We currently have the three following scenarios in front of us tonight:
-They clamp her tomorrow and the fenestration starts to work. Ava reabsorbs her CSF on her own. Hooray! We get to go home! Unfortunately, this is not going to happen due to the amount of CSF still draining every day.
-They clamp her tomorrow and the fenestration does not work. They unclamp her and the CSF fluid looks the same as today, which is yellow with a little blood in it. This means that they can move forward with the cysto-peritoneal shunt either tomorrow afternoon or Thursday.
-They clamp her tomorrow and the fenestration does not work. They unclamp her and the CSF fluid looks worse than it did today, which means it has more blood in it. They will then continue to drain the CSF fluid for a few days. The cysto-peritoneal shunt will be put in closer to Friday or Saturday.
The update tonight to all of you is that Ava is going to have a fourth brain surgery this week. A permanent cysto-peritoneal shunt will be surgically implanted into Ava’s brain. Ava will be “shunt-dependent” the rest of her life. The success rate of a shunt is around 95%. The 5% of failure has to do with second or third surgeries to fix the original shunt. The original three risks of surgery exist for Ava as well. So, your prayers for Ava are much appreciated.
Dr. Foy explained the procedure tonight. There will be three incisions. They will go through the original burr hole on the top of her skull. They will put the shunt in either the subdural space or the arachnoid cyst space. Dr. Foy said that he would put the shunt in the subdural space. He doesn’t know how his partners would perform the surgery. Dr. Lew and Dr. Kaufman are on tomorrow through the weekend. This plays into our story because Dr. Foy is leaving town again tomorrow night, Wednesday night.
I like Dr. Foy. He has been with us since Ava’s first surgery. He came back from vacation yesterday. Ironically, his time off was spent in Barrington, IL. His parents live there and his grandparents live across the street from Ava’s preschool. Dr. Foy saw the Fourth of July fireworks and the parade that Ava would have seen if we were at home. He went to the Barrington High School. He graduated high school in 1995. We really like him. It was good to see him again. We were hoping that he would perform Ava’s fourth surgery.
The other big issue with Ava is a feeding tube. The battle over the feeding tube has turned into a very interesting story. I will keep it short. The neurology fellow ordered it on Saturday night based on the influence of the nursing staff. Ava wasn’t meeting her goals. The nurses’ side of the story is that Ava has lost two pounds in two weeks. I fought them on it because I thought it would be very traumatic for Ava to have another tube coming out of her body, especially her nose. Ava doesn’t handle the wires/tubes very well. The feeding tube would be in her throat every time she swallowed. I was trying to protect her from more pain and more traumas. I have seen Ava only eat a little every day. I have seen her only drink a little every day. I know she is losing weight. However, I know she will eat when she feels better. I just know it.
When I asked both of the neurologists, they told me the same thing. They absolutely do not think Ava needs a feeding tube. They feel that Ava still has her “fight” in her. They feel that Ava “looks good.” They feel that a feeding tube will be detrimental to her recovery. They feel that she will make up her calories/weight as soon as she feels better. Dr. Foy looked into the nurse’s eyes tonight and said, “Ava does not need a feeding tube. If Ava was my daughter, I would not do it. It is not the battle we need with her right now.”
The feeding tube debate will continue tomorrow and through the week. We will see how she does before her surgery. Justin and I definitely do not want to jeopardize her health/weight in any way. As her parents, we will continue to listen to both sides of the argument and will try to make the best decision for Ava.
Ava’s nights have been worse than her days. So, Ava’s journey continues tonight and again tomorrow.
Thank you for all of your prayers. We truly appreciate every single prayer for her full recovery.
Thank you for spreading Ava’s story to your friends and family.
We want her headaches to go away.
We want her to start to heal from all of the pain she has endured over the past two months.
We want her to eat and drink again.
We want her to be healthy again.
We want to see her laugh and tell jokes again.
We want to walk out of this hospital with Ava sooner than later.
We want to see her play with her brother and her sister again.
We want her to play with her new puppy that she got for her birthday.
We want to go home and be a family again.
Love,
Gina
July 12, 2013
Ava is having her FOURTH cranial surgery. My emphasis is on the “4th” part. The start time will be between 12p-1p. It will be performed by Dr. Lew, the same neurosurgeon that performed her arachnoid cyst fenestration.
Ava was clamped on Wednesday at around 10:30a. Her headaches returned immediately. She had an eye appointment around 12:30p. Justin and a nurse, Michelle, took her to the appointment. She had to sit up in a wheelchair the entire time. She was in so much pain. Justin said that she handled herself well but it was breaking their hearts to watch her try not to scream in the waiting room. They brought her back to the room around 2p. As the afternoon went on, Ava’s headache episodes increased in pain and in frequency. She was hooked up to a machine that monitored the intracranial pressure. Justin’s eyes were glued to it. The machine was similar to the machine women are hooked up to during pregnancy/delivery.
By 6:00p, we still hadn’t seen a neurosurgeon. Ava’s pain was unbearable. We were in tears. It was so hard to watch her. She was screaming and rolling around in pain. Heather, our nurse, had paged the neurology team several times. We knew Dr. Foy was leaving the hospital at 4p. So, we figured we had to focus on Dr. Lew now. The nurse paged the neurology team again. Finally, one of the neurology fellows came down to talk to us. Justin and I were very upset. We asked her why Dr. Foy or Dr. Lew or even Dr. Kaufman did not come down to talk to us today about Ava’s drain being clamped. Today…of all days…was extremely important for Ava. They had clamped her drain and she was absolutely miserable from the intracranial pressure. The neurology fellow explained that the plan was for Ava to be clamped for two days. Justin and I almost screamed out loud, “ARE YOU CRAZY?!!!”
Ava’s pain level was so high between 3p-6p. Justin explained his case for unclamping the drain perfectly. I tried very, very hard not to scream, “Just unclamp the drain now!”
The neurology fellow went back to talk to Dr. Lew and Dr. Kaufman. The nurse received a phone call around 6:20p that she was to unclamp the drain. Ava’s pressure was released and she was immediately feeling better. We made it through the night.
We were expecting surgery yesterday. The nurse woke us up and told us that Ava was not to eat anything. We were ready for the surgery. Today was going to be the day. We were going to move on the path towards recovery. Ah. What? Not the day.
Dr. Lew came down around 10a. He spent a good amount of time with Justin and me. He pulled up Ava’s last MRI. We walked through the cysto-peritoneal shunt procedure. There are several decision making factors with installing the cysto-peritoneal shunt.
In addition, Dr. Lew had some concerns about whether the shunt would eliminate all of her headaches because her intracranial pressure spiked while she was clamped. The average was around 15, which is normal for a person. However, Ava was still having headaches. Justin pointed out two things for Dr. Lew to consider. The number one thought was that during the first of the two drainage procedures, we drained her at between “0 and 5 pressure” – and she had immediate and lasting relief while the drain was in. During the draining process for the third procedure, fenestration, we drained her at between “20 and 15” on the scale. She did not have the same type of relief. Justin asked Dr. Lew if it was possible that Ava might be extra-sensitive to pressure, even “normal pressure.” She has an autonomic neuropathy that plays into the sensitivity of sound, touch, headaches, etc. He thought it was a very good point, and thus instructed the nurses to reduce her pressure to” 5” and see if she would have a “good” day. If she did, then that would give him more confidence about putting in a “high-drainage/low pressure valve”. Per Dr. Lew, it seems that Ava needs a lot of drainage for relief.
The other decisions to make are as follows:
The location of where they put the shunt is very important. Option one is in the subdural space. Option two is in the arachnoid cyst space. Both Dr. Foy and Dr. Lew agree that they want to put the shunt in the subdural space. The main reason is because of the relief she felt when the drain was in on top of her skull in the subdural space. The second reason is because Dr. Lew does not want to reopen the space where the incision was for the fenestration. The recovery would be a lot more painful for Ava.
Another decision is what type of catheter to use. As of now, it’s a “5” option or a remote control option. The benefit of the remote control option is that you can change the shunt drainage at any time. The downside to the remote control catheter is that you have to reprogram it after every MRI. Justin and I need to learn about how hard is it to reprogram the catheter. The remote control catheter is a lot more money, which should be covered by our insurance without a problem. However, the doctors have to justify it as the only option.
So, we will discuss further with Dr. Lew today as to which catheter he will use. I think we are all pretty comfortable with the “5” catheter valve – meaning a high drainage/low pressure valve.
Ava has not yet had the feeding tube put in. They weighed her again yesterday. She was 41 lbs. She’s lost 4 lbs in two weeks. However, she ate an entire hot dog, bowl of strawberries, edamame and a yogurt smoothing last night for dinner. The argument is actually getting uncomfortable with the nurses and the nutritionist. Dr. Lew reiterated again today how he would not put in a feeding tube. The neurology team feels strongly that Ava will start to eat once she feels better. Thank you for all of your opinions on the feeding tube. It has been helpful to hear your stories.
On top of everything else, our family has been working with the Children’s Hospital of Wisconsin Diagnostic Odyssey team on our autonomic neuropathy issues. We have been meeting with Dr. Bordini since our first admission here on July 13, 2013. He has taken our “case” on as his own. He met with a panel of top doctors here at the hospital on our behalf yesterday. The panel includes neurology, dermatology, genetics, academics and several other specialists. They plan on taking skin and muscle samples from Ava today during surgery. They are taking a 1cmx1cmx1cm cube of muscle from her thigh and several circular punctures on three of her limbs. They are taking a skin/scalp sample to freeze. They are taking blood to freeze as well. They want blood samples from all five of us to freeze too. We are thankful to have doctors on the case.
Eventually, we hope the doctors here will help with Ava’s autonomic dysfunction, which she suffered from much more during the colder months. I seem to be suffering the most from it right now. However, I feel horrible that Ava has to be the one having samples taken from her. I wish they would take the samples from me. I wish I could be the one enduring all of her pain.
Her samples are going to be used by many specialists in hope that they help all of us. How much more can a child handle? A woman I have never met from Chrissy’s church, Ellen, gave me a necklace a few weeks ago. It has a saying on the charm, “I can do hard things.” The other day when Ava was supposed to do something that was difficult for her, she said, “I can do hard things. I can do hard things.”
I said, “Oh Ava. You can do very hard things.”
Today, Ava will do a lot of hard things.
Ava will have four major incisions:
-The top of her skull. This is for the shunt into the subdural space.
-The back/side of her neck. This is to link the shunt/tube and the tube running to her peritoneal cavity.
-Her left side of her abdomen. This is where they put the catheter into the peritoneal cavity for drainage
-Her thigh muscle. This is for the autonomic neuropathy biopsy
Thank you for all of your continued love, support and prayers.
Again, I want to pray for the following…
We want her to make it through surgery today without any complications.
We want the shunt to work and for her headaches to go away.
We want her double vision to go away.
We want her to start to heal from all of the incisions today as well as from all of the pain she has endured over the past two months.
We want her to eat and drink more on her own.
We want her to be healthy and strong again.
We want to hear her sing again.
We want to see her beautiful artwork again.
We want her to smile again.
We want Ava to walk out of this hospital herself sooner than later.
We want to see her play with her brother and her sister again.
We want her to play with her new puppy that she got for her birthday.
We want to go home and be a family again.
We want to go home and be a family again.
We want to go home and be a family again.
July 13, 2013
Thank you for all of your prayers yesterday. They definitely made a difference. Ava made it through both surgeries with no complications.
Since Ava had two different surgeons perform each one of the surgeries, she was back in the surgical unit for a long time yesterday. We didn’t get out of recovery until late last night. Dr. Lew, the neurosurgeon, felt that the surgery went well. However, only time will tell.
We were pleased that they did not have to do the third incision in her neck or on the base of skull. However, I wasn’t really prepared for the long incision on the top of her skull. She now has two long incisions on the left side of her skull. She also has the incision in her left side of her abdomen, where they put the catheter into the peritoneal cavity for drainage. This seems to be the most painful part of the surgery for her right now. They went through her stomach muscles. Whenever she talks or moves, she is in a lot of pain.
Her thigh hurts her as well. They took out a good amount of muscle for the biopsy. The surgeon was thoughtful. He did it up as high as he could so her shorts would cover the scar. The three biopsies on her arms are upsetting to her. They are very visible to her. They covered all four incisions from the biopsies with a certain “glue” to seal the wounds. They are all purple.
We are still waiting to see if her intracranial pressure stays within the “normal” range. She is hooked up to the machine again that monitors her intracranial pressure. She has only been lying flat in her bed. She doesn’t want to be elevated at all. It hurts her abdomen. The night was very heartbreaking. She was grabbing on to me for life. She made me hold my hand on her chest all night. The nurse kept her on morphine and Toradol all night and will continue to do so through the next 24 hours.
Dr. Kaufman is the covering neurosurgeon over the weekend. He’s the Head of the Department of Neurology. He’s a tough guy. He came in and was a little impatient with Ava. I didn’t feel that he had the right to talk to her like that since he hasn’t been involved in any of her surgeries. We didn’t say anything. He told her that she had to sit up in the bed. No questions. Done. She cried but the nurse and I elevated the bed a little bit. I explained to him that the Pediatric General Surgeon that performed Ava’s biopsies told us that she wasn’t going to be able to walk for a few days with the incision in her thigh. Dr. Kaufman agreed. He said that she doesn’t need to get out of bed yet, she just needs to sit up today.
We are pretty confident that the shunt will help Ava and we will be home soon. However, we do not want to rush it. We hope they keep the monitor on her for a few more days. We want to be absolutely sure that she doesn’t start to have intracranial pressure headaches. As much as we want to go home, we will push for the monitor to stay on until we feel comfortable with her activity level. She has always felt better lying flat in her bed. We want her up and walking for periods of time before we take her off of the monitor.
We thank you again from the bottom of our hearts for your love, concern and prayers. Thank you for all of your emails/texts/phone calls yesterday. We appreciate every single one of them. We will update you again in a few days. Hopefully, the update will be that we are going home.
Thank you for taking the time out of your busy lives to read our updates. I know everyone has a lot going on. So, thank you for being so concerned about Ava and our family. Your love, support and prayers have helped us get through this very difficult journey.
Love,
Gina
July 16, 2013
Well, here’s the update we have all been waiting for…
Ava is being released from the hospital today.
I’m very excited. However, I am also very apprehensive. Ava is still not eating or drinking very much at all. She definitely has not hit her “marks” as far as the nurses and the nutritionist are concerned. She also has a lot of abdominal pain that is not from her incision. We do not know what is causing her pain right now. We took an X-Ray yesterday and it showed that her bladder was distended. It also showed all of the tubing in her abdomen. It was fascinating to see what her abdomen looks like now. Unfortunately, Dr. Lew did not feel it was necessary to do a CT or an ultrasound of her abdomen today, although it would have made me feel more comfortable taking her home. I guess I have to trust him. I just spoke to the psychologist. She said, “Ava’s journey has never been easy as far as the doctors are concerned. It’s probably not going to start being easy now.”
Dr. Lew felt that Ava will do better out of the hospital. He watched her hide under her blanket today when he talked to her. He watched her cry when we asked her questions. I think he thinks that she is really damaged and needs to get out of this room to heal. I agree with him very much. However, I’m nervous about her abdominal pain as well as her diet. She also has headaches that she says are now a “four” as well as her double vision. Dr. Lew said that she will continue to have headaches. We just have to watch them closely. He said to bring her back if her abdominal pain or her headaches get any worse. We will also have to bring her back if she continues not to eat or drink. We have follow-up appointments with Dr. Lew, the pediatric ophthalmologist as well as the psychologist set for July 24th.
So, Ava and I are headed to Madison for a few days. We have to pick up Oskar and Lucy. We are looking forward to a little rest and time with everyone there. Justin is in Las Vegas for the rest of this week. We hope to be together again as a family in Barrington by the end of the weekend.
I hope and pray that Ava transitions well and does not have any more pain in her head and/or in her abdomen. We hope that she starts to eat and drink as soon as we get to Grandma’s and G-Daddy’s. We hope she is able to start to heal emotionally as well as physically.
Thank you again for all of your prayers, love and support during this very difficult time.
Ava’s journey will continue…but it will not be from these four hospital walls.
I am walking out of this hospital with my Ava today.
I will get to put all three of my children to bed tonight.
Thank you God. Thank you everyone.
Much love,
Gina
Shortly after Ava was released from the Children’s Hospital of Wisconsin, a rainbow magically appeared without rain the morning of July 19, 2013. Ava made a wish for her recovery and we told her it was the Angels welcoming her home. We call it Ava’s rainbow. 
July 30, 2013:
Dear Family & Friends,
I wanted to give you an update on Ava’s wonderful progress.
We had her follow-up appointment back at the Children’s Hospital of Wisconsin on July 24th. Her MRI showed that the midline shift looked improved and that there was a little less CSF fluid on the left side. This meant that the shunt was working well. Dr. Lew was very pleased with the way the MRI looked as well as the way Ava smiled. He said, “It’s the first time I’ve seen her happy.”
He was also very pleased that Ava had not had any headaches since her fourth brain surgery. He told us that he would see us again in three months for another MRI and follow-up.
Although Ava was not having any headaches, she continued to have intense abdominal pain. She made us a little nervous at the follow-up when they weighed her. Unfortunately, she had lost another three pounds since she was discharged. Dr. Lew still felt that he would not need to place a feeding tube in her nose. He continued to believe that she would do better outside of the hospital walls. I was nervous about it. I felt that I wasn’t going to be able to get her to eat at home either. We decided to give her two more days. Ava was due back at the hospital on Friday, July 26th, for another appointment. At this time, we would weigh her again. If she was still losing, then we would have to start the feeding tube.
Fortunately, something clicked and she started eating on Thursday night, the 25th. Since then, she has been getting better and better every day. She is eating new things and enjoying food again. Her energy is returning and she is getting stronger. Her sense of humor is back and she is telling jokes again.
Ava is very knowledgeable about everything that has happened to her. She is still dealing with night terrors/nightmares. She has been reliving her time in the hospital through them. Her other concerns and worries are mostly centered around any future medical procedures. She can explain her shunt and can draw the picture of it perfectly. She describes it better than the doctors did to me. With each new day and each new experience, we see her confidence building. She is still timid around new people or new situations. We keep planning “fun days” instead of “doctor appointment days.”
We are still waiting for Ava’s results on the skin biopsies and the muscle biopsy for her autonomic neuropathy. Dr. Bordini, the autonomic specialist, helped us deal with a severe rash Ava had once she was out of the hospital. It was triggered by the heat and/or sun. It went away after two days of being inside in the air-conditioning. We are still waiting for our official evaluation with the autonomic clinic for our entire family. We hope to have this done within the next month.
I am trying to work through my pain of having to witness my daughter suffer through excruciating pain for three months as well as almost being blind and dead. We still have questions regarding the neurosurgeon and neurologist at Lutheran General in Chicago, IL. We do not understand why they did not feel it “necessary” to rescan Ava. They both could have stopped her suffering numerous times in their emergency room. We are also disappointed in the doctors at The Mayo Clinic in Rochester, MN. Dr. Fischer, her autonomic specialist, told me to take her for a walk to stop her headaches. Dr. Mack, her neurologist there, put her on medication without scanning her again. Although I am very happy and relieved to have her home, I cannot get the images of her suffering out of my mind. Over time, I am praying that I am healed as well.
Justin and I feel so very blessed to have her home and for her to be doing so well. Our families feel like the “dark cloud over their heads” has disappeared as well. I attached a picture of her for you all to see. We had to cut her hair. She loves the “new look.” She seems a lot older now and wiser too. We feel like she’s aged beyond her six years old birthday in June.
Thank you again for all of your prayers. Your prayers truly helped save my daughter’s life. I am forever grateful to all of you. Thank you for being there for our family.
Love,
Gina
September 16, 2013
Dear Family and Friends,
If you haven’t heard already, our family is moving to Salt Lake City, UT.
Justin was offered the position of Assistant General Manager with the NBA Utah Jazz. Justin accepted the job under his professional name, Justin Zanik. He started working there on September 3rd. He is very excited about the opportunity to work with the organization. The Utah Jazz actually waited for Ava to get out of the hospital before they offered Justin the job. Family is a priority to them. They wanted to be sensitive to the situation.
I will be flying with the kids this Tuesday, September 17th. Our wonderful nanny is coming with us for two weeks. Justin’s mother will be there for five days to help us unpack.
We found a house to rent while we decide when and if we are selling our home in Barrington, IL. We were all set to sell it, however, I’m having a hard time letting it go…
Ava is thriving and blossoming into a fun loving, bright little girl. She was able to attend her North Barrington School for the past few weeks. They welcomed her with open arms. She loved every minute of going to school there.
All three kids are excited for the move. Ava is going to start a new school on Monday, September 23rd. It’s called The Waterford School. It’s an independent school providing a liberal arts education. Oskar and Lucy will start preschool soon after we get there. Justin is checking out a school for them this week.
I wanted to say a special thanks to my family in Madison. My Mom and Dad have taken care of Oskar and Lucy the majority of the summer. Thank you so much for being there for us. Thank you also to Shannon, Holly, Chad, Tristan, Sunny and Mike for encouraging me to be so positive about the move. Their support has helped me through this transition. I love you all very much. I’m going to miss you more than you know.
I also wanted to extend an invitation to everyone to visit! We have two extra guest rooms. Come visit Utah
Love,
Gina
October 13, 2013
Dear Family and Friends,
I wanted to share an article that was printed today in The Salt Lake Tribune.
Link to the article: Before he joined Jazz, Justin Zanik first helped daughter
We feel very blessed to have met Bill Oram. He interviewed Justin for an article about his new position with the Utah Jazz. When Justin opened up about the summer he experienced with his family, Bill was fascinated. He felt there was such a strong family-oriented story to be shared with his readers. Bill and I met for the first time last Saturday, October 5th. However, I feel like I’ve known him for years. Bill read all of my updates and my journal entries.
He was also able to spend some time with Ava. After he read my journal, he said that if he didn’t already know the outcome of the story….that Ava is doing great….he would have had a very hard time finishing it. His heart felt for all of my readers, my family and friends that survived the summer along with us. He said it must have been such an emotional roller coaster ride for everyone reading the updates. Thank you for reading my updates. Thank you for being there for Ava as well as for Justin and me.
The even better news that I wanted to share is that we had Ava’s follow-up this past Friday, October 11th. She had a MRI and an appointment with Dr. Lew. Her MRI looked even better than in July. The midline shift is almost completely gone. The fluid on the left side is even less than before. The shunt is doing its job perfectly right now!
Dr. Lew was able to see Ava dressed in her pink and wonderful style. The picture is included below. He was able to listen to her talk on and on about moving to Utah and her new school. Ava was excited to tell him, “I’m BACK!!”
She was also THRILLED that he told her she should be able to snow ski in Utah. I wasn’t expecting him to say that she could snow ski already. I’m not sure how I feel about it yet!?!
One little story I would like to share. Ava was in the ER at Primary Children’s Hospital last weekend, October 5th & October 6th. After the scrimmage for the Utah Jazz, Ava went home. She wasn’t feeling well. Our nanny and close friend, Brittany, was with the kids for the evening. She called at about 10p and said that we needed to come home right away. Ava was screaming that her head hurt on the right side and that her spine hurt in her neck. Hearing her scream again brought the fear back in all of us right away. I called Children’s of Wisconsin and spoke to the resident. He told us to take her to the local ER immediately.
We met with their many ER doctors. They came in and explained that “they have never actually seen a shunt placement like Ava’s before in their ER.” They wanted to call their Neuro Team. Meanwhile, her CT and X-rays came back just fine. They were baffled as to why she was in so much pain. Justin stayed with us until 4a. Unfortunately, Ava and I spent the night in the hospital. All the memories came flooding back. The four little walls. The getting awoken throughout the night from nurses coming in and out. An IV in Ava’s hand. The beep, beep, beep. Ava had night tremors again while in the hospital. Her moaning and screaming all night was horrible to listen to again. The nurse kept coming in. I’m pretty sure it was from her high fever.
The next morning. The nurse practitioner came in and asked if Ava had been tested for Strep. I informed her that they did a lot of blood work, CT scan and X-rays but no strep test. They had swapped her nose but no strep test. Turns out…..Ava had strep. I don’t think I have ever been so happy to hear the diagnosis STREP before. There was nothing wrong with her shunt! YEAH!! Let’s get Ava on antibiotics and get out of here!!!
When I met with Dr. Lew, I asked him why the ER doctors told us that they have never seen a shunt placement like Ava’s. He explained that usually a shunt is in the ventricles or in the subdural space. Ava’s is in her cyst area. Sometimes, the brain can expand once the fluid is gone and push the shunt up against the skull. Ava’s shunt is protected by the arachnoid cyst. Is an open space that cannot be pushed over by the brain? I asked him, “So, are you saying that the arachnoid cyst is helping the shunt and keeping it safe?”
He replied, “That’s exactly what I’m saying.”
I asked, “Why didn’t you ever tell us that before?”
He explained, “We didn’t know if it was going to work.” He gave me a smile.
I really thought my last update was going to be my last one to all of you. However, I had to share the great news from Friday as well as the wonderful article written about our family in The Salt Lake Tribune.
Oh how far we have come…
Our Ava is doing very, very well….
I told my mother on Friday afternoon. “Mom. This is the first day that I have felt true relief and happiness in a really, really long time. I have nervously and anxiously been waiting for this follow-up appointment. Seeing Ava’s MRI today made me so happy.”
Thank you again to everyone that has been there for us through this journey. I’ve used that word, “journey”, many times over the past year. I was not asking for this journey, I didn’t know it would last so long and change so many times. The journey from Ava’s trauma to Justin’s success to Ava’s recovery to my continued struggle to keep my head above water to our move to Utah…wow…
A few of the lessons I have learned this year….
Listen to your gut
You know your child more than anyone else
Don’t take no for an answer just because they are wearing a white coat or scrubs
Pray to God
Have others pray to God for you
Open your heart to the generosity of complete strangers
You will be blown away by how much strangers can touch your heart
Ask other people about their story
Everyone has a story
If you listen deeply to their story, their gift to you is beyond what words can explain
Love your family
Love your friends
Love yourself
Breathe
Big breaths
Even bigger breaths when you feel like you are going to lose your mind
I can do hard things
We all can do hard things
Much Love,
Gina
April 27, 2014
Dear Family and Friends,
I wanted to update you again on our family. I have had so many wonderful people reaching out to us recently to check in on Ava, Oskar and Lucy. Thank you. We appreciate it very, very much.
I have been slow to respond to a lot of you. I apologize. It’s been such an overwhelming couple of months again.
I realize that everyone has their own story of what’s going on in their lives…
So, I appreciate the time you take out of your busy lives to read my updates.
I love hearing from all of you. I appreciate every single email response I receive after I send out our updates. You all help me to continue on this journey. Thank you.
March 20, 2015
I haven’t written an update in my journal in the last year that just focused on Ava. I have been updating through my blogs here on the RUN website. Please read the blogs to continue Ava’s journey. She is still undiagnosed with a genetic dysfunction, autonomic neuropathy, small fiber polyneuropathy, arachnoid cyst, EOE and shunt-dependent. She still suffers from heat/cold intolerance, fatigue and pain. Lot’s of pain. (Same as of June 15th, 2019)
Much love,
Gina
Read More of the Szajnuk’s Journey
Learn More about #UndiagnosedDay, Undiagnosed Rare Disease Day
ABOUT RUN
RUN stands for the Rare & Undiagnosed Network. We’re a group of advocates, patients, families, researchers, and healthcare providers who share the same mission and vision: To empower rare and undiagnosed patients and their families with genomic information and community through advocacy, networking and support.
Contact information:
Gina Szajnuk (Zanik)
Co-founder and Executive Director
E: ginaszajnuk@gmail.com
C: (310) 883-4353
To Read: “What is the hardest part of being undiagnosed?” by Gina Szajnuk
To Read: “Undiagnosed is a Diagnosis” by Ava and Gina Szajnuk
To Read: The Szajnuk Journey