What is congenital hyperinsulinism? We have no idea what is was until our son was diagnosed with it.
We are not medical professionals and we cannot give precise definition of the disease. All we know in layman terms is that the pancreas produces a lot of insulin. This disease is the total opposite of diabetes.
We know from reading materials that this is a rare disease and it can happen to 1 in 50000 babies.
Our son was diagnose with hyperinsulinism from birth. He did not show any symptoms of hyperinsulinism. The first time we had discovered that he had a problem was when he slept all day and not crying or waking up for milk. We felt something was a mist and we rushed to the hospital. At the hospital his blood sugar was 1.1 mmols/l . The doctor had to immediately set an Glucose IV on him. Our son had been in and out of the hospital 2 times for this condition where the doctors at the private hospital were unable to determine what our son was suffering from. He was ok for 4 months and the same problem happened again. He was drooling from his mouth so we are sure something was wrong . We went to a local clinic to check his blood sugar and this time it was 1.9 mmols/l.
I was struggling to find out what was wrong with our son, but since I was a diabetic I when to see my Endocrinologist for my regular check up and explained the situation to her. My doctor immediately made arrangements for me to send my son to a government hospital which specializes in this type of illness.
We immediately admitted our son to the hospital and stayed there for a couple of days. During this time the doctors took some blood sample when he had hypoglycemia. Since my son's blood sugar was stable during the couple of days when he was admitted we were allowed to go home. The doctors explained to us that they are unable to determine if the low blood sugar problem was at the beginning, at the peak or decreasing.
The doctors told us to monitor him and his behavior. The very next day after we got home he had a seizure like symptom and we immediately sent him back to the hospital. Luckily the doctor who treated us was on duty and he carried out further testing. Our son was admitted for 2 months after that day.
The doctors at the government hospital who are specialists in this field knew what they were doing and conducted the tests and blood tests systematically. After taking our blood and our son's blood samples for further genetic testing to determine if our son had hyperinsulinism. While waiting for the blood test results, he was treated with diazoxide to try to control his blood sugars. Besides that our son needs 2 hourly feeding with an addition of corn starch to help supplement his low blood sugars. Hence the intense regiment had to be continued hoping that his blood sugar will be controlled.Unfortunately, diazoxide had no effect on him. Our son still had hypoglycemia. The doctors that had an infusion pump of glucagon fitted to his belly and the needles need to be changed every 6 hours. It broke our hearts to see that our son was under so much pain.
After 4 weeks, in the hospital, there was not much improvement on this blood sugars as he still hypoglycemia. The doctors told us that the last line of defense is to use Octreotide ( Subcutinous Injection) to try to control his blood sugars. He will need 4 injections a day and we will see how he progresses. We thank you god that Octreotide manage to control this blood sugars. But this has to be done together with the intense 2 hourly feeding, blood sugar testing every 2 hours and of course in between the feeding corn starch has to be given.
Once this regiment proved to be efficient without the glucagon infusion pump, we were allowed to go home finally after 2 months in the hospital.
We had to continue with the intense regiment of continuous feeding and 4 injections a day for the next 6 months until the doctors told us that his condition had much improved and we were allowed 3 injections a day instead of 4. That was the greatest news of our lives at that time.
Subsequently, we had reverted to 3 injections of Octreotide a day. Our son's condition was some what manageable. He is quite active during the day as he is learning how to crawl and walk. At times he will hypoglycemia 5 to 6 times a day. My wife have to be exceptionally careful in giving him glucose. If he gets too much glucose our son will have diarrhea. Since I have to work during the day, my wife is the most important person to me. She is the best wife and mother anybody can have. She is very patient, creative and she knows the patterns of my son's behavior. Although my wife and I do not get much sleep, but we remained positive and strong to take care of our son. We also have a 5 year old daughter who is schooling and we need to care for her as well.
The 3 injections a day and multiple 2 hour feeding when on until the next medical check up arrived. We had a long discussion with the specialist and ask the doctor to be realistic on how long will his condition last. The doctor can only stay that with his current condition, with his routine and injections, there may be a possibility his condition will be more manageable after 3 to 4 years. We totally understand that this condition will be very difficult for us to continue this way. I am determined to find a cure for my son, since part of the diagnosis led us to find that his congenital hyperinsulinism has a high possibility of a focal lesion. Once the focal lesion is removed, he will be cured.
I started to search the internet for more information on which hospital and who are the specialist who can treat and cure this disease. I found 4 countries that have this expertise, USA, Israel, UK and France.
The first option was to write to USA since I have lived there before and am pretty familiar with the place. After 2 months of continuous correspondence with Philadelphia, we have finally got the cost of treatment and surgery. The estimated cost of treatment and my stay there in Philadelphia will cost us a whopping RM 1 million. The reason is the patient will need time to recover from surgery and stabilize his/ her blood sugar before they can return home. So we are looking at 2 months to 2.5 months stay in Philadelphia.
The first option was no successful, as we cannot possibly afford RM 1 million for our son's treatment.My heart just shattered as first I do not know how to revert to my wife. She will be so disappointed and 2nd I feel so helpless. I saw a possible cure for my son but I cannot sent him for treatment because it is too expensive. After a day or two and getting myself together, I told myself I still have other options, what about other countries? I spoke to my French boss about my son's condition and he helped us to write and email to France at the Necker's children hospital for more information. The hospital did not reply but my French boss( Mr Erwan Beros) best friend's sister works in the hospital. So the doctor who was in charge of Congenital hyperinsulinism replied to our email. Due to our son's congenital condition we could not get any medical insurance hence the doctor in France recommended a few doctors in Europe in order for us to compare the cost of medication.
One of the doctors that we contacted was in Germany. Prof Dr Klaus Mohnike a renown pediatric endocrinologist from Germany replied to our email. He also introduced Prof. Dr Winfried Barthlen who is a renown pediatric surgeon for hyperinsulinism. Shortly after receiving the email from Prof Dr Klaus, Prof Dr Barthlen called us. He is the most caring and the friendliest doctor you can speak to. He introduced himself and explained the medical costs to us. To our surprise, we can actually afford the medical costs and expenses. The next question to us was are you willing to fly half the world to come to Germany?
I told the surgeon that if you can confirm that the medical costs are affordable, we are on our way to Germany.
I went home to discuss this with my wife before long we were preparing for our trip to Germany. We had mixed feelings during the preparation of the journey as one we were scared and do not know what to expect? Secondly and most importantly we know that we have found a cure for our son. Of course, our journey will not be complete without lots and lots of love and support from our family, friends and colleagues.
It took us approximately 3 weeks to prepare for the trip.
Finally the day came for our departure to Germany. First stop KLIA, it was really a challenge as our son hypoglycemia at the airport. As usual, we had to prepare his glucose to be fed through his nasal tube.His nasal tube inlet valve had failed. So we rush him to the KLIA clinic below to see the doctor on duty. Unfortunately, the doctor does not have the right size nasal tube for replacement. He manipulated the tube and finally we manage to give our son the glucose he needed. We thought that was the end of our misery but unfortunately it was only the begining.
We were almost the last passengers to reach the boarding area, but we were allowed to board the plane since we had an infant. 2 hours after taking off, it was time to feed our son. We were12 feet in the air, traveling at 830mph, our son's nasal feeding tube failed again. There was no way the plane will turn back. We were still 10 hours shy of reaching Frankfurt. I have tried multiple ways to try to feed him through the tube but the milk and water was no going in. We were panicked. Likely our boy is good he was sleeping throughout the flight. I tried to suck and blow through the tube and if needed I will drink the milk and blow it into the nasal tube( to some it may be disgusting, but I don't have a choice) before I attempted to do that I manage to find an angle were I can syringe the mile through this nasal tube.I fed our son using this way for the next 10 hours en route to Frankfurt.
The journey to Frankfurt was not an easy one. As soon as we have reached Frankfurt the first thing we have done was to look for the ticketing counter to get the earliest flight to Berlin. During this time our son had shown some of his hypoglycemia symptoms. I was at the ticketing counter while my wife checked his blood sugar. It went down to 1.9 mmol. I had to run in between the ticketing counter to my son since I had to give him glucose to increase his blood sugar in the shortest time possible. The nasal hose is still having the problem and I had to do what I did in the plane to manipulate the nasal tube in such a way that I can inject the glucose through the nasal tube. After 30 minutes, my son's blood sugar stabilized at 4.0mmol. We manage to get the earliest flight to Berlin at approx 9.45 am. We reached Berlin at approx. 11.45 am.
Upon arrival, we quickly check our son again and so far his sugar is stabilized. We got our luggage and when straight to the taxi stand. We tried to flag down taxis but we were not lucky to get one because it the the law in Germany for taxis to have baby seat to carry baby passengers. After one hour at the taxi stand finally a good taxi driver stopped and agreed to pick us to the hotel since he had a child seat in his taxi.
The hotel staff were very friendly and we had checked into a room with no problems at all. We quickly left the main bags in the hotel and packed whatever we needed to go to the children's hospital in Berlin Lichtenburg to spend the night before the PET CT scan.
Upon arrival at the reception, we have language difficulty as non of the nurses speak English but there was a ER doctor who spoke English to assist us. We explained that the main concern is that our son's nasal tube has malfunctioned and that we were not able to give glucose or give him milk through his tube. The doctor immediately organize the nasal tube and of course his Octreotide injections.
After one and a half hours at the reception, we were admitted to a room and had our son's nasal tube replaced. We were delighted that our feeding problem was solved. Since none of the staff spoke any English I spent the night at the hospital with my wife and son. We took turns to take care of our son, as you may understand my wife is totally exhausted from the journey and taking care of our son.
The next morning, we met the anesthetist who with an English translator advised us on the procedures before the PET CT. He set an IV line on our son then we were on the way in an ambulance to the PET CT facility.
During this time, before we proceeded to the PET CT, we met our room mate for the first time. They were a family from Israel. They have a baby girl who had the same CHI disease as our son. So we were there at the same time to do the PET CT and then go to Greifswald for surgery.
At the PET CT scan center, We traveled in an ambulance for approx 15 minutes to reach PET CT center.
The anesthetist let us to a few corridors and we have finally arrived. This is the first time we saw the PET CT machine. It is like a CT scanner but what is special is the 18 flouro dopa nuclear substance used to scan the pancreas. This substance had to be made hours before the PET CT with the life span of only 4 hours. After 4 hours this nuclear chemical cannot be used. As soon as our soon was on anesthesia, we saw a nurse rolled in the 18 fluro dopa. This nuclear substance is not made in Berlin but it is made at a special facility some 300 kms away from the center. All we can say is that, a lot of work, preparation and timing needs to be correct for this PET CT scan.
The good news is that we met Prof Dr Barthlen for the first time. We were so relieved and composed as we knew that we all are in good hands. The moment you meet Prof Dr Barthlen, you will find him to be very compassionate, warm and most importantly you feel as if he is a very good old friend. We think that is the most important feeling you should get when you are in a foreign country with no friends or relatives. Prof Dr Barthlen always kept to his word when he says that he is at the PET CTs. He is always there to answer any questions that you may have and he has great patience. During the PET CT we also met Dr Wolfgang Mohnike the Director for the PET CT facility, Prof Dr Klaus Mohnike, the CHI Pediatric Endocrinologist.
After 2 hours of PET CT, our son was finally out of the treatment room and there were some preparations made before we can return to the hospital. Meantime, we met Dr Klaus Mohnike and we had a short discussion about the PET CT. He said we found the focal lesion at the head of the pancreas. We were dumb founded with the discovery as we were hoping for the focal lesion to be at the tail of the pancreas. Based on my readings and findings that I know, if the focal lesion is on the head and body of the pancreas the surgery will be quite complicated. Prof Dr Klaus Mohnike and Prof Dr Barthlen said, don't worry, rest well tonight and tomorrow morning, you will make your journey to Greifswald. This where Prof Dr Barthlen will be expecting us.
We returned to the hospital worried, but we know for sure that we are in the best hands. So since hospital room had the other Israel baby with her mommy, I cannot stay too late at the hospital. So I left my wife and our son at the hospital while I return to the hotel to pack everything to so that we can go to Greifswald the next day.
I tried to get rested for our trip to Greifswald. Early next morning I had all the luggage ready and headed straight to the hospital in Berlin. Before long we were outside the hospital, thinking on how we would proceed to Greifswald. The weather was quite cold that morning with slight drizzle. We made our way by foot to the nearest subway train station. Since it was our first time at the train station with many stairs to climb, We do not know where or how to purchase the train tickets since it was through machine and it only displays German. Further down the long train station I found the information counter whom the lady was quite helpful to assist me in purchasing the tickets for us. We boarded the train to Berlin main station where we were suppose to change trains to get to Greifswald. Upon arrival at the Berlin main station we have trouble locating the ticketing counter for which the train we need to take. The train station is huge. While we were running around the train station trying to look for the ticketing counter, it was time to check our son's glucose again. It was no surprised that he had hypoglycemia again ( 3.0mmol). We had to stop to make glucose and fed him through his nasal tube. Finally we found the ticketing counter and purchased the tickets where the cashier said that the train will leave in 10minutes at platform 5. We ask the counter where is platform 5, she said it should be downstairs. We rushed downstairs and luckily the platform we were at is platform 5 otherwise we would have missed the train. The train ride to Greifswald was a comfortable one. It was a very comfortable 3 hours train ride to Greifswald with lots of scenery and post war buildings which fascinated us.
After the 3 hours train ride we finally arrived to Greifswald. It took us 15 minutes by Taxi to reach the Greifswald hospital where our son will have his surgery. The moment we received the reception, we mentioned our son's name ( Ashton) immediately the nurses knew who we were. We were escorted to the surgery ward for surgery patients. As soon as we had settled in our room, Prof Dr Winfried Barthlen came in to the room. He said that everything will be alright, just relax and try to make ourselves comfortable and he will see us in the next 2 days to discuss Dr Wolfgang Mohnike, Prof Dr Klaus Mohnike and his findings of the PET CT in Berlin. The next 2 days was pleasant settling into Greifswald after our long journey from home. We rested pretty well for the 2 days and bought Ashton around Greifswald town.
Finally, Friday came and we had our meeting with Prof Dr Winfried Barthlen. He is very warm, kind and smiling cheerfully as usual. He welcomed us to his think tank room. He did not bit around the bush but he went straight to the point. He said that in the PET CT images in Berlin indicated that the focal lesion is located at the head of the pancreas. We were totally dumb founded as the worst just had happened to us.
We were hoping that the focal lesion was at the tail of the pancreas where the surgery will be less complicated. Prof. Dr Barthlen said, although it will be a difficult surgery but he will try his best to remove the focal lesion. He mentioned that he was worried that there is one lesion on top of one another meaning 2 focal lesions on the head of the pancreas. He was worried that the 2nd lesion is pressing on the main duct that transport the gastric juices and other enzymes to the stomach. He also mentioned that if the duct is damaged during the surgery he will have to perform a Rouen Y technique. Basically it means that the damaged part of the pancreas will be removed and to join the 2 remaining parts of the pancreas he will need to take part of the intestine to join the end of the pancreas. This means that our son will have 2 highways to transport the gastric juices or other enzymes to his stomach. Prof Dr Barthlen also mentioned that his record for surgery is 11.5 hours but our son may take up to 13 hours or more. We are totally shocked, worried and blanked.
After 10 minutes when we got our senses together we agreed to the surgery since we know that our son is in the best hands. I signed all the papers ( in German) which I did not understand. So the surgery was scheduled on 13th NOV 2011. The night before the surgery Ashton had to be transported to the ICU in order for them to set a IV line at his inner thigh. The needle was easily 3 inches long and there were at least 3 supply tubes to supply our son with sodium, glucose and other nutrition. My wife stayed with our son the whole night until the next morning I joined my wife in the ICU. Prof Dr Barthlen came in the ICU at approx 6.40 am, he looked at Ashton and said that we will do the surgery today. Shortly after he left the nurses came in to the room and started their preparation to transport Ashton to the Operating Theater.
It was the longest lift ride of our lives. Ashton felt some was a mist and he held on to his mommy very tight. As soon as we arrive to the holding room, ( the place you say good bye before the door to the Operating theater) It was very difficult for us to leave Ashton but we had no choice. We cried our lungs out and I slowly held my wife out of the holding room. We were instructed by the Prof Dr Barthlen not to stay in the hospital but to go to town. He took my mobile number and he said that once he is done, he will call me immediately. We were reluctant to leave but eventually we walked to Greifswald town. To be honest, we were walk to town like zombies because we left our souls in the Operating Theater. We were like 2 crazy chinese with no direction on where to walk or what to do. It was the longest day of our lives. We looked at our phones and watches very 5 minutes. It was forever for 5 minutes to pass. We met the Israel baby who were with in Berlin, came from Berlin to have the same surgery as Ashton but Ashton was the first to go into surgery. We stayed and chat with the family since we had no other thing to do. This helped to kill the time. Soon it was getting dark so we headed back to the hospital, worried since we did not get any calls from Prof Dr Barthlen. We ran straight to the ICU to ask one of the nurses if she had any news of our son. She said that he was still in surgery. We kept pestering the nurses every 5 minutes until their patience were thin as paper. We remember at 4.45pm German time my phone rang. It was none other than Prof. Dr Barthlen.
The first thing he said was can you come to my room now? My wife and I was already running before I could reply him. He is always smiling when he saw us and he mentioned that the surgery took 8 hours instead of 13 hours. There were no complications and he took 25 biopsies on the pancreas. Prof Dr Barthlen said indeed the focal lesion is located at the head and body of the pancreas. But during the biopsies he could not find the active tissues at the front of the pancreas as suspected. The pathologist said that the tissues in front of the pancreas are healthy. So Prof Dr Barthlen, operated on the surrounding tissues to access the rear portion (back side) of the pancreas. Hence he flipped the pancreas like a fish and he found the focal lesion and removed it. In total he removed 10% of Ashton's pancreas and that the remaining 90% of the pancreas was healthy tissues. Prof Dr Barthlen and his team was comfortable of this results and closed Ashton up.
We could not help to go give Prof Dr Barthlen a big hug. He said that Ashton will be in the ICU for a week and he will be taken care off by all the nurses 24/7 to monitor his blood sugar. He also mentioned that the first 3 days he will be in the world of his own since he was highly sedated. This is a major surgery hence the doctors need to ensure that the baby do not feel or sense any form of pain. Our son had at least 3 machines working on him at anytime. His blood sugar stabilized immediately after the surgery. He remained at 5.5 mmol throughout the week which was very good. We were advised and worried that he may need insulin jabs after this surgery as his blood sugar can sky rocket. We thank God and Prof Dr Bathlen that everything is good.
We stayed in Greifswald for the remaining 2-3 weeks until Prof Dr Barthlen came to tell us that the final pathologist report came out to say that Ashton's remaining 90% of his pancreas are healthy. This calls for a celebration and he said that we can go home in a weeks time. I made all the necessary changes to our flights almost immediately. We also need to thank the hospital staff and a doctor to assist us in planning our trip back from Greifswald to Frankfurt.
We arrived from Greifswald to Frankfurt after an 8 hour train ride and we stayed the night at Frankfurt.
The next day we were early and full of energy as we are going home to Malaysia.
Finally, we arrived home after a 12 hour flight. This is our son's hyperinsulinism. He is cured from this disease. He is going up to be normal kid.
We would like to encourage parents whom their children are diagnosed with congenital hyperinsulinism not to give up hope. We would also like to encourage the people that had took interest and time to read our blog who know people or children who may have congenital hyperinsulinism to pass the message around that there is hope for this disease. It is a very rare disease that affects 1 in 50000 babies. We are praying that all the babies who are born in this world are healthy but if for those who are affected please don't give up. Only the parents of children with congenital hyperinsulinism will understand the regiment of continuous feeding every 2 hours and Octreotide injections 4 times a day, is a very pain staking task. Many parents will agree that without exceptional patience, faith and understanding it is very difficult to carry on.
We are very lucky to have met and contacted Prof Dr Winfried Barthlen and Prof Dr Klaus Mohnike that have found a cure for our son. So we are encouraging patients in ASIA or Malaysia who wish to find a cure for Congenital Hyperinsulinism to come forward after reading our story. Parents please understand the initial difficulties of the journey, but once you are there everything will fall into place naturally.
Once parents have decided that this is journey is for you, please feel free to contact us at the email address in my profile. If we can do it, we are sure that other parents can do it. We as parents will go to the end of the world for our children. We cannot offer any financial help, but we can share our experiences on how to get there to cure your child. For those parents who do not have any information about Congential Hyperinsulinism in Malaysia, now you have a contact and we can provide you with all the information we have and share our experiences with you. Remember DON'T GIVE UP and NEVER LOSE HOPE. There is a will there is a way.
Please note that I am not a certified doctor or an expert in congenital hyperinsulinism. I cannot offer any medical advise. Please ensure that all the diagnosis and findings are done at a certified hospital by specialists in Malaysia.
Thank you very much to everyone who has taken their time in reading this blog.
Be safe, go well and live life to the fullest.
