Hannah Stempfel
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Hannah was born on 12/5/2000 at St. Johns Mercy Medical Center in St. Louis Missouri by means of a C-section. My pregnancy was very normal with no complications. Immediately after birth, Hannah was given some oxygen and was rushed away to the NICU. What I did not know, was that this was the beginning of our nightmare.
Later that day, they had to intubate her in order to keep her SATS and CO2's within normal limits. At this time, there were still accessing her and could not tell us what was wrong. Two days and some routine tests later, Dr. Denis Altman (pediatric neurologist) came to talk to us and told us that he thought she had Congenital Central Hypoventilation Syndrome
(CCHS). This is a disorder of the central nervous system in which the autonomic control of breathing is absent or impaired. A CCHS child's respiratory response to low blood oxygen saturation (hypoxia) or to CO2 retention
(hypercapnia) is typically sluggish during awake hours and absent (to varying degrees) during sleep and serious illness or stress. This syndrome is very rare with only 200 cases worldwide. My initial thought was it was totally impossible for Hannah to have this and asked what evidence he had to base this diagnosis on. There were two other children in St. Louis born with this syndrome and both were born at St. John's under the same group of doctors. We asked about the outcome of these children. One lived and one died. Not the statistics that we wanted to hear.
The only way for them to diagnosis CCHS was to do every test imaginable on Hannah and if they found nothing, then CCHS would be the official diagnosis. The phrase that they used was "If it walks like a duck and sounds like a duck, then it must be a duck." My husband and I were totally against putting her through this at first, but after discussions with all the pediatric neonatologist's and nurses, we did not have much of a choice if we wanted to find out what was wrong with Hannah and bring her home.
Hannah was very strong and active and kept pulling her breathing tube out. On December 12th, she was trached. I was finally able to see my little girls face. She did however still have her NG tube, but I could now hold her more freely without the fear of pulling something out. After her trach surgery, they tried numerous times to wean her off the vent but were unsuccessful.
In early January, the doctors at St. John's consulted with Dr. Pamela Schuler of St. Louis Children's Hospital. Upon meeting her, I immediately liked her. Her main goal was to get Hannah stabilized on her vent and send her home. On January 12 Hannah was transferred to St. Louis Children's Hospital so that Dr. Schuler would have a better watch and say on her care. Things were fine the first week, then it was like another nightmare. Every resident that walked into her room ordered tests after test (MRI, spinal tap, etc.) I refused everyone of them and said that she already had them done at St. Johns and for them to obtain a copy of the results. I finally told Dr. Schuler what was going on in her absence and things got straighten out…fast.
In late January, they let me try bottle feeding Hannah. I noticed that her SATS would drop into the low 80's while she was feeding. The nurses kept telling me that this was totally normal. I paged Dr. Schuler that evening and asked her about this. She ordered a swallow study for the following day. Low and behold, Hannah was aspirating. She recommended that in order to get her home, she should have a flundo to fix the aspirating and put in a G-tube. We agreed and on February 5 Hannah had surgery.
The next 3 weeks were touch and go. They were doing daily blood gases on Hannah, and her CO2 were always out of range, either too high or too low. First it ended up being respiratory acidosis. They made some vent changes and corrected the problem. The following week, it ended up being metabolic acidosis. It was like a roller coaster, one day she would be fine, the next day she would throw herself back into the acidosis. Dr. Schuler and I both felt that we could deal with this at home and the longer she stayed in the hospital, the higher chance she had of getting RSV, etc…
Finally on March 3, 2001, two hospitals and 3 months after her birth, Hannah finally came home with an ambulance escort. The day was a huge celebration along with tremendous amount of fear. Never in my mind did I ever think that I was going to have to take care of a child on a ventilator. The first few months, I let the nurses run the show and took lead from them. I learned so much from them and gained so much confidence from their encouragement.
Five months after bringing Hannah home and getting her settled in, I figured that it was time to find out more about
CCHS. Through a support group, I found another family that lived in Missouri, about 2 hours from us. I called them to see who their doctor was. Their daughter was a patient of Dr. James Kemp at Cardinal Glennon Children's Hospital. I called him the following day, and he was glad to hear from me. Come to find out, he knew all about Hannah but due to medical guidelines, could not call me. I was very impressed by what he knew about
CCHS. He has 4 other patients with this syndrome. Although we hated to switch doctors, I knew that it was the right thing to do. I did not hesitate and made an appointment to see him the following week.
Dr. Kemp was very impressed with Hannah. He was concerned that she required 24 hour ventilation. The majority of CCHS patients only require ventilation while asleep. Because his other 4 patients were only vented at night, he suggested that we go to Rush Presbyterian Hospital in Chicago to see Dr. Jean Silverstri and Dr. Debra
Weese-Mayer. They have the largest registry of children with this syndrome. We made an appointment to see them in September. Hannah had just turned 9 months old.
During our first Chicago visit, sleep studies were performed on Hannah, and her diagnosis was confirmed. We also found out that only 8% of CCHS patients require 24 hour ventilation, and Hannah was one of them. They sent us home with a game plan. At first, we were very hesitant. The new orders made us monitor Hannah's SATS and CO2 every hour, and make vent changes accordingly to keep her SATS and CO2 within the limits that her doctors ordered. Since Hannah has no lung problems, her doctors felt that she could be adequately ventilated and oxygenated with the vent, not O2. As reluctant as we were, it worked.
Everything fell into place after this. After Hannah turned 1 year old, we pulled her G-tube out. She continued to eat and drink well. In February, 2002, I asked our DME company to get us the new LTV 950 ventilator. Since Hannah was very active, we needed to be more mobile. They refused. After a few phone calls and a letter from her doctor, we had her new vents in March. We loved the new vent and started to travel to more. It was just what the family needed.
In April of 2002, we headed back to Chicago for Hannah's yearly checkup. They were very impressed with her. Since she would still require 24 hour ventilation, they suggested the
"Phrenic Nerve Pacer". This is an implant that would stimulate her to breath, without the vent. Very portable. The receiver is small enough to wear in a child size backpack. She was tested and we were told that she was a good candidate for the surgery, but we just got the new vent and were so mobile anyways, we didn't want to put her through the surgery. Needless to say, 6 months later when she started walking, I regretted not having the surgery done. In July, 2003, we scheduled the surgery. She came through with flying colors and healed wonderfully. It has always amazed me how much she has been through and how fast she bounces back. We went back to Chicago in September 2003 to have the pacing unit activated. It worked great for the first 2 days. It was totally AMAZING and overwhelming to see her run the hallways without a vent. On the third day, we ran into some problems, and upon testing the unit, it was not functioning properly. They did not know why, and would only be able to tell during another surgery. We racked our brains, having to make a decision within the next day and decided against it. We had been through so much that year already, and could not put her though surgery again. We decided to wait until the follow year to fix the pacer. She would just go home on vent. Before we even told the doctors our decision, they paged us. They got the results of her routine holter and found a 4 second delay in her heart. The said that the phrenic nerve pacer was no longer a priority, and we had to deal with the heart issue now. This was common among CCHS patients, but we just always hoped it would not affect Hannah. I called Dr. Kemp that night and talked in length with him on what to do. We didn't know if we should bring her back to St. Louis for a second option. He told me that he trusted the doctors judgment and that this was not something we should take lightly. We met the following day with the doctors and agreed to have a cardiac pacemaker implanted in Hannah. She had surgery the following Monday and was discharged on Wednesday. We spent 2 weeks in Chicago and was not looking at going back anytime soon.
Well here we are today. Hannah is going to start preschool in April. Until then, she is receiving PT, Speech and OT at home. Her preschool teacher also comes to the home once a week. She has grown so much during the past few months, I can't imagine what changes to expect when she goes to school. We have scheduled our next Chicago trip for May 2. We feel that Hannah needs to be as mobile as possible. Even though she is still considered 24 ventilator dependent, she has been tolerating using trach collar that dispenses 24% oxygen during the day for the past 8 months. She has not had another episode with her heart and has not used her cardiac pacer yet.
We have definitely had our ups and downs over the past few years. Like many others, we have been affected by the nursing shortage and the struggle of having to work full-time and take care of the household, as well as nursing nightmares and hospital screw-ups. Early on, everyone would tell me that God wouldn't give me something that I couldn't handle. In my mind, I kept thinking "what did I do wrong" or "why couldn't he have given this problem to someone else". Now I see things differently. Yes, I am still agitated that this happened to me, but I think it has made me a stronger person. I figure this is Gods test for me…I just hope that I am passing.
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Karen Stempfel
