Tristan, 17 years old, 15 years old at time of Best practice: “From the start Tristan couldn’t suck a bottle so we had a hard time getting him to eat. We used bottles that were flexible and would basically squeeze the bottle to help him drink it. Eventually we found out Tristan was tongue tied so he had surgery to have that clipped.”– Danette
Eva, 6 years old, 2,5 years old at time of Best practice: “For many years Eva suffered from severe feeding intolerance, which resulted in marathon hospital stays. She had episodes of pain and non-stop vomiting, gagging, retching for days. Her GI system would shut down and she needed IV fluids. Eva’s doctors developed a program entailing a specialized feeding plan and schedule, GI motility medications (preventative measures to support GI function), and a recover plan when Eva’s GI system stopped and pain started.
Feeding Plan and Schedule – Eva was put on Peptamen Jr. 1.0 formula. We fed at a slow rate, continuously over many hours. At night Eva’s GI function slowed down, so we gave four hours of clear fluid (pedialyte) to flush her system at night.
GI Medications for Motility and Preventative Cyclical Vomiting –
Augmentin – Eva began taking a small dose of Augmentin in the morning and at night. The Augmentin is given one hour before feeds are started. Augment increases motility in the small bowel.
Liquid Senna – Eva gets liquid Senna twice a day to stimulate her GI motility.
Miralax – Miralax is mixed with Pedialyte, which Eva receives at night as noted above.
Periactin – Periactin is given twice a day to prevent cyclical vomiting episodes. We give Periactin with the Augmentin one hour before feeds are started.
Medications used during GI shut down and/or Cyclical Vomiting/Pain spells-
Ibuprofen, ondansetron, and ativan is given for pain and/or nonstop vomiting. We give these medication quite close together to take the pain away, and we give every 4-6 hours depending on the severity of the episode.
Iberogast – is a herbal liquid solution made in Germany that helps sooth the stomach. Eva’s GI doctor has given us the verbal approval to use this solution. It is great for stomach pain, bloating, nausea, gastro-intestinal cramps. We order it online via Amazon. It is quite pricy, but worth it!
Eva is not able to tolerate feeds during a GI shut down cyclical vomiting/pain cycle, so we put her on pedialyte, and gradually add formula into the pedialyte until she recovers.”– Sheri
Talynn, 3 years old, 2 years old at time of Best Practice: “Talynn has many issues with motility and vomiting. Right before her first birthday, she got a gtube and nissen fundoplication. This stopped the vomiting but then Talynn started retching ALL the time. By the time Talynn had turned 2, we had tried every type of medicine the doctors suggested and did multiple pH probe tests. I had seen where “…” [parents] were trying various things to improve this. “…” [One] suggested Cyproheptadine and “…” [another] suggested Blended Diet. We did both March 2015 and since, Talynn is able to go to the bathroom on her own and has not had a gag or retching episode.”– Taylor
Okke, 5 years old, 5 years old at time of Best Practice: “Okke has problems with his PEG-tube. The inner bumper was almost complete mucosal overgrown. Pushing and pulling did not dislodge the bumper. A gastric tube was passed through the external PEG into the gastric cavity. This catheter was attached to the external PEG tube with a clamp. The intragastric part of the catheter was grasped with an endoscopic forceps. With gentle traction the bumper was dislodged, and after removing the clamp and catheter, the PEG could be extracted without complications.”– Sünne
Florian, 2 years and 7 months old, 2 years old at time of Best Practice: “Vomiting caused by constipation caused by poor digestion. Florian started vomiting when he was just 4 weeks old whilst he was having expressed breast milk via ng tube. When he switched to formula the vomiting continued and he struggled to put weight on. He had a gj tube fitted when he was 6 months old and the retching and vomiting (this time of bile) continued. For the next year we were admitted into hospital on several occasions with vomiting and not being able to tolerate feeding. He was xrayed each time to check for obstruction (none) and jej placement (fine) so was put on gut rest on dioralyte for 24-48 hours until he could tolerate diluted feed and then full feed. Sometimes he needed a dose of diazepam to relax his bowel as it would spasm. Eventually the doctors concluded that constipation was behind the vomiting and we started on a series of constipation medication that unfortunately made him crampy and uncomfortable without really helping the constipation. What felt frustrating was that we were given constipation meds without a proper explanation as to why he was becoming constipated. His poo was always soft to the point of runny so I couldn’t understand why movicol (a stool softener) would help. The answer was often to do with poor gut motility and a neurological issue but I thought back to when Florian was about 4 months old and how you could feel him bearing down when he wanted to poo so wondered what had changed. After a lot of thinking and reading and speaking to other members of the group I wondered whether he was struggling to digest his formula and that’s why his gut wasn’t moving well. He’d been on a prophylactic dose of trimethoprin for about 18 months due to a urine infection so I wondered whether this had impaired his digestion too. We started giving him homemade chicken broth to help heal his gut and a probiotic supplement to help replenish his gut bacteria. We also came off all the constipation meds and started doing water enemas if he didn’t poo. It’s not perfect yet but we’re heading slowly in the right direction.” – Alice
Stephanie, 4 years old, 2-4 years old at time of Best Practice: “Prone to throwing up when she is sick or stressed Have had difficulty gaining weight as a result. Can’t be “solved” but we are making progress by recognizing early signs of stress or incoming sickness and adjusting feedings accordingly. When sick we switch to pedialyte for first 24hrs then run pump at half rate. We also know not to do a feeding before appointments, haircuts etc to minimize episodes of throwing up. We have figured out continuous night feeds work best for her so she gets most of her fluids at that time.” – Kathy
Isabelle, 8 years old, 3 years old at time of Best Practice: “Almost every other day she would throw up a little. Then about twice per month she had horrible episodes. She would vomit until everything was gone and then retch from the bottom of her toes for hours. She would stop and then start seizing. Then go back to retching. This continued usually for about 24 hours. We switched her diet to the introduction to GAPS diet. And slowly added calories. She hasn’t thrown up (except for illnesses in the past year). And she can take on almost twice the volume she used to, with no difficulty. Part of this is easily digestible food, and part is the kefir and cultured food.” – Julie
Gracelynn, 18 months old, 12 months old at time of Best Practice: “It was a constant battle to meet the calorie intake recommend by the nutrionalist. Gracelynn was being feed with a pump by G-tube. Since the pump go’s by a pre set volume and rate there was no telling if she was full. This caused consistent bouts of vomiting. Which would lead to aspirations and respiratory issues. We found that doing bolus feeds with a large syringe and gravity instead of the pump gave us the freedom to vent her stomach of any gas and also gauge if she was becoming overfilled before getting to the point of vomiting.” – Judy
Gracelynn, 18 months old, 12 months old at time of Best Practice: “We found that if Gracelynn didn’t have a daily BM after 2 days she would vomit. Formula alone was not enough fluids for her. Adding 3 bolus water feeds (one 1st thing in the morning before formula) 1 mid-day and 1 last thing before bed.” – Judy
Kennedy, 9 years old, 6 years old at time of Best Practice: “Failure to thrive.
Missing her milestones and not gains weight. Gtube and nisson [helped].” – Darla
Lorelei, 20 years old, 11 years old at time of Best Practice: “Lorelei was diagnosed with [Chronic] Pancreatitis in March 2006. She had a large cyst on her pancreas that caused fluid around her lungs to back up. Every time we would try to feed Lorelei her enzyme levels would go back up. The answer finally came with a J tube. Her surgeon had to place a red rubber catheter directly into her. Her scoliosis was too severe for them to pass a GJ tube. Her specialized food also helped her. She gets Vivonex everyday using a feeding pump.” – Erica
Lorelei, 20 years old, 11 years old at time of Best Practice:“Through blood work we were able to tell Lorelei was very low on B12 [B12 Deficiency]. Lorelei has been getting B12 injections every month since September 2014. It has made a difference on alertness. She does not sleep during the day as much.”– Erica
Okke, 5 years old: “Okke is born with a double-sided cleft lip and palate. Okke is depending on tube feeding from birth. Until he was 4 months old, he has drunk with a Habermann bottle small quantities himself. After his suckling reflex was gone, he was no longer able to drink himself. When Okke was three months old, he started to vomit regularly. First one or two times a day, then several times a day (on bad days 20-30 times per day). He vomit his entire stomach content.
Okke had a nasogastric tube (ng-tube). When he was 2 years and 2 months old, he got a gastric tube (g-tube). That has no significant influence on the vomit. We had no problems with the g-tube. After three years, the g-tube (held by an internal bumper in the stomach) replaced by another g-tube (held with a balloon in the stomach) because the bumper was growing together with the stomach wall.
Causes that worsen the vomit were upper respiratory infections and urinary tract infections. Stress and pain are a trigger for the vomit. Also constipation affects the frequency of vomiting. Therefor he got Forlax junior (macrogol).
We have tested different tube feeding (Nutrini Peptisorb, Nutrini, Nutrini Low Energy Multi Fibre). That had made no difference. In phases where Okke didn‘t tolerated no nutrition, we gave him 1 to 2 days just electrolytes or water. Then we gradually dilutes the nutrition with water until he tolerates undiluted tube feeding again. We are convinced, that Okke need fewer calories as a ‘ normal ‘ child with a similar age and weight. (He got 780 kcal per day at a weight of 19 kg and gains weight. Advised are 1500 kcal!)
We had a feed pump. Thereby we were able to customize the walk-in rate. On good days Okke got 4 to 5 times a day power supply (100-200 ml/h). On bad days got Okke proportionally less food, more water/electrolytes at a lower walk-in speed (50 ml/h). All that has not been able to stop the vomiting but can reduce.
Okke was never able to eat solid food. We give him small quantities to let him taste food.
To soften the side effects of vomiting (upcoming stomach acid), Okke got antacid. First Zantac (ranitidine), than Nexium 10 in form of powder (sachets) (esomeprazole), that helped better.
Zofran (ondansetron), a cure for severe nausea, had no significant impact.
Mortilium (domperidon), a medication to stimulate the movements of the stomach and the first part of the intestines and speeds up the passage of food from the stomach to the intestines, has had no effect. On the contrary, Okke got painful abdominal cramps and because he was still very young (4 months) probably epileptic activity.
When Okke was 3 years old, we were being swimming with him a number of times. After swimming he has not vomit for around two days.
We always had the suspicion that one cause was neurological. First Okke got Fenobarbital when he was 9 months old. Because we saw no real improvement over time, we have phased out this medication gradually. That lasted a year and until Okke was 3 years and 7 months old. In this time it went with Okke very well. The vomit was less severe (1 – 4 times a day) and sometimes he not vomit a day. Only on days where he was sick, he has vomit more.
Unfortunately, his epilepsy got worse again after a year. When Okke 4 years and 7 months old, he has gotten Depakine (Valproic acid). And voilá, after two weeks he did not vomit anymore. We could hardly believe it. BUT! First we haven’t taken it so seriously. But his epilepsy was despite increasing the dose progressively becoming worse. Okke had never large tonic-clonic seizures (just one time). At last he had constantly epileptic activity even that he could no longer sleep. We have apparently found a drug that (as a side effect) stopped the vomiting BUT his health and well-being went dramatically down. Although all neurologists said: “The vomiting is not neurological. The vomiting can only have an organic cause.” I think I don’t have to tell, that the vomiting has made me desperately. We have learned to deal with. We knew when Okke vomit and had a spit cup on time. So we had an overview of his moisture balance. But the epileptic activity made me ANXIOUS!
Unfortunately we had no chance to build off this epilepsy medication. Okke died with an age of 5 years and 8 months after a surgery on his tonsils … We always have tried to make life for Okke as comfortable as possible. That keep in avoiding stress and pain. Lots of rest, especially at mealtime. Oblique posture when lying and sitting with meals, as it can. Okke has always had much fewer calories than a nutrition centre advises. And I think the most important thing: much, much love!” – Sünne
* Although children who are diagnosed Bohring-Opitz Syndrome (BOS) have a lot in common, each child is different and unique and the variation in level of illness from one child to the next can be great. Please keep in mind that Bohring-Opitz Syndrome is very rare and there is still little known about the clinical management of children with Bohring-Opitz Syndrome. These Best Practice results, experienced by parents, caregivers and doctors worked for this specific child. Therefore we think it is important to present, to discus and to consider these findings. However, we hope that more BOS children can benefit from these experiences. It is important to realize that given the complexity of the illness of each child a Best Practice of one patient may not help another BOS patient. Each child should have an individual care plan, which is designed to meet the unique needs of the patient. The plan should be routinely followed, monitored, and adjusted as needed.
The material and content on “Bohring-Opitz Syndrome – A worldwide exchange of information and awareness” is for information only and not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.
Bohring-Opitz Syndrome 