Congenital HI Kids .org: Resources

We give Collin cornstarch with a glass or two of whole milk each night before bed. (1-2 heaping tblspns per glass). To my knowlege, medical professionals are not certain why, exactly, the cornstarch works to stabilize blood sugar in kids with HI overnight. I know that cornstarch is NOT to be used in diabetics or children under a certain age (1 year?). The cornstarch looses effectiveness for helping to maintain bloodsugar when heated or added to liquid with sugar. It can also be added to feeding tubes. Be warned, that you should clean up or drink the stuff ASAP-- it can get messy!

D10 or D20 is used by IV when Collin gets sick (especially stomach flu) to keep his blood sugars safe. We have to take him to his physician &/or the hospital for this to be administered.

Glucagon is used for emergencies when Collin's blood sugar drop dangerously low & he becomes unresponsive.

Jennifer

State Specific Assistance

State Health Agencies(50 states)

http://www.fda.gov/oca/sthealth.htm

Health Coverage for All Children Campaign

http://www.childrensdefense.org/helping-americas-children/childrens-health/health-coverage-for-all-children-campaign/

AAP Children's Health Topics: CHILDREN WITH SPECIAL HEALTH CARE NEEDS

http://www.aap.org/healthtopics/specialneeds.cfm

Speech And Language

Speech and Language Developmental Milestones [NIDCD Health Information]

http://www.nidcd.nih.gov/health/voice/speechandlanguage.asp

Speech and Language Milestones for Babies and Children

http://www.childrensdisabilities.info/speech/language-milestones.htm

Free speech therapy tips for children with speech, language delay

http://www.leomagan.com/

How to Teach a Child With Speech Delay

http://www.ehow.com/how_2069849_teach-child-speech-delay.html

Family-friendly supports for communicating with picture symbols and pecs

http://www.givinggreetings.com/

Special Feeding Needs

So Your Baby / Child Needs To Be Tube Fed

http://infantrefluxdisease.com/infant_acid_reflux/tube-feeding.php

How to Care for a G-Tube

http://www.ehow.com/how_2053455_care-gtube.html

Percutaneous Gastrostomy Tube Care, Cincinnati Children's Hospital Medical Center

http://www.cincinnatichildrens.org/health/info/abdomen/home/perculator.htm

Catheter Care

How To Care For Your Foley Catheter Care Guide Information

http://www.drugs.com/cg/how-to-care-for-your-foley-catheter.html

How to Care For Your Catheter

http://alzheimers.about.com/od/caregivers/a/catheter_care.htm

Peripherally Inserted Central Catheter

Pediatric Peripherally Inserted Central Catheter (PICC) Line Insertion

http://www.cpmc.org/learning/documents/piccline-ped-ws.html

Carbs & Diabetic Menu

Low-Carb Diet - Kids and Low-Carb Foods

http://pediatrics.about.com/od/obesity/a/708_lowcarbdiet.htm

High-Fiber Low-Carb Food List - Fiber Counts in Low-Carb Foods

http://lowcarbdiets.about.com/od/nutrition/a/fibercounts.htm

Counting carbohydrates - includes list of foods with carbohydrates

http://findarticles.com/p/articles/mi_m0675/is_n1_v10/ai_11729520/

Carb Counting- Healthy Eating - American Diabetes Association

http://www.diabetes.org/for-parents-and-kids/diabetes-care/carb-count.jsp

Children with DIABETES - Readers' Favorite Recipes

http://www.childrenwithdiabetes.com/recipes/

Diabetic Menu Planning

http://www.your-diabetes.com/diabetic-menu.html

Complete Diabetic Menu

http://www.diabitieslife.com/diabetes/diabetes-diet/food/complete-diabetic-menu.htm

Our Support Groups

CongenitalHIkids(Yahoo)

http://health.groups.yahoo.com/group/CongenitalHIkids/

Causes on Facebook | Congenital Hyperinsulinism

http://apps.facebook.com/causes/241732/52311804?m=0409b04a

Causes on Facebook | Congenital Hyperinsulinism (HI) (CHOP)

http://apps.facebook.com/causes/318635/70733659?m=fb5a6ed7

MySpace.com - Congenital HI kids.ORG

http://profile.myspace.com/index.cfm?fuseaction=user.viewprofile&friendid=400137770

Medical Id Tags

Medic Alert Symbol : In My Jammies

http://www.cafepress.com/inmyjammies/3105766

MedicAlert® Watch

http://www.medicalert.org/Watch/

Medical Alert Bracelets

http://911medalert.com/?gclid=CI-zqfy-0poCFRJexwodkQMTFg

Sharps Compliance

http://www.sharpsinc.com/

Words to Google

Sur1 & Sur2

Diagnostic Algorithm

Differential Therapy

Pancreatic Imaging

Biochemical Background for Pancreatic Imaging

Correlation of Histology and 18F-DOPA-PET

Imaging of the Pancreatic Focus

Congenital Hyperinsulinism

Medications (The information on this site is on this link below CHOP)

http://www.chop.edu/consumer/jsp/division/generic.jsp?id=71065

Medical Treatment
Includes feeding, as well as drugs that block insulin secretion.

Feeding
Oral: babies may need to be fed a specific amount of sugar or other carbohydrates on a certain schedule to avoid low blood sugar. These feedings may include breast milk or infant formula, glucose water (also called dextrose water), and in older infants, cereals or bread and other starches. Feedings alone are rarely sufficient treatment.

Tube: involves an infusion of dextrose water or formula into the stomach. The tube is passed down the nose and into the stomach. For long term treatment, a gastrostomy tube can be placed directly in the stomach. Tube feedings may be used when the baby can not take in enough at feeding times. In this situation, formula can be infused over a short period of time through the tube. Babies can easily become dependent on the tube feeding and have difficulty eating by mouth. Careful attention must be paid to avoid this dependence. Some babies who take enough formula by mouth still need a continuous infusion of nasogastric dextrose to keep the blood sugar normal. This type of tube treatment does not typically interfere with the baby's feeding ability.

Drugs
Diazoxide (Proglycem): oral medication given 1-3 times a day. Works on the SUR to block the release of insulin. In some types of HI, diazoxide works well , in others it is ineffective. Common side effects of Diazoxide are:

* Hypertrichosis - Extra hair growth on the body (eyebrows, eyelashes, back, and arms) which goes away months after medication is stopped.
* Fluid Retention - Some babies require daily diuretic medication for a short time to avoid fluid retention.

Octreotide (Sandostatin): given by injection 3-4 times a day. It works by decreasing the secretion of insulin, however, it does not work directly on the SUR. In place of shots, octreotide may be given as a continuous infusion using an insulin pump. Common side effects of Otreotide are:

* Short-term - transient diarrhea
* Long term - possibility of gallstones. Suppression of growth hormone is a theoretical concern, but is usually not a problem.

Nifedipine and Verapamil: calcium channel blockers. Work by blocking calcium from entering the beta cell and therefore, stopping insulin secretion. Unfortunately, they are not often successful in preventing low blood sugar.

A common side effect of Nifedipine and Verapamil is low blood pressure.

Glucagon: an injectable drug that briefly raises the blood sugar by releasing sugar from the liver into the bloodstream. Used as an emergency drug to quickly raise the blood sugar for 1 hour when the baby has low blood sugar but cannot feed. Can also be infused slowly into the vein while the baby is in the hospital for several days while tests are performed and treatment decisions are made. Chronic treatment of HI with glucagon at home is not currently feasible.

A common side effect of Glucagon is a brief period of nausea and vomiting.

Surgical Treatment
Removal of part of the pancreas (partial or subtotal pancreatectomy). Surgery is performed when medical treatment fails to prevent low blood sugar. The type of surgery depends upon the type of HI. With Diffuse KATP HI, a 95 % pancreatectomy is commonly performed. With Focal KATP HI, removal of only the diseased focal area of the pancreas is attempted.

Surgical treatment can result in:

* A cure of focal HI if the focal area was completely removed
* Stabilization of blood sugars with or without additional medical treatment
* Continued low blood sugar
* High blood sugar (diabetes)

For the first few days after surgery almost all babies have high blood sugar and may require insulin. The high sugar immediately after surgery is a result of the stress of surgery and anesthesia. It is not an indication of the baby's ultimate need for insulin or any other treatment. The outcome of surgery can be tested after the infant has recovered from the operation and is eating normally again.

Genetic Defects and Diazoxide
Hyperinsulinism is caused by many different genetic defects. The following are the three most important in relation to Diazoxide.

Potassium channel defect
The most common form of hyperinsulinism is caused by a defect in the potassium channel (KATP channel). In many cases this channel is missing and in other cases it is there but not functioning properly. Diazoxide acts on this channel to prevent insulin release. Therefore, in cases where this channel is missing, Diazoxide will not work.

In cases where the channel is present but not functioning properly, Diazoxide may work a little or not at all.

Hyperinsulinism/hyperammonemia syndrome
The second most common cause of hyperinsulinism is the hyperinsulinism/hyperammonemia syndrome, in which the gene defect has nothing to do with the potassium channel, but instead interferes with the metabolism of glutamate in the cell, causing excessive insulin secretion. Children with this syndrome all have normal potassium channels and thus Diazoxide works on this channel and prevents insulin secretion.

As a result, most patients with glutamate dehydrogenase hyperinsulinism will be responsive to Diazoxide.

Glucokinase hyperinsulinism
The third type of hyperinsulinism, which is extremely rare, is glucokinase hyperinsulinism. In this condition the potassium channel is also functioning normally but the defect is at another site in the cell. Therefore, in this condition, Diazoxide, by acting through the potassium channel, works very well to prevent hypoglycemia.

Diazoxide
Diazoxide is an oral medication that can be given anywhere from q day to tid. The dose is generally prescribed based on body weight and ranges from 5-15 mg/kg/day. As the dose gets higher, the side effects of the drug become greater. The main side effects are initially fluid retention (increased swelling of the feet, hands, and face), increased weight gain from excessive water being stored in the body and in small infants, this can cause heart failure due to fluid overload.

A later side effect is the increased growth of hair on the body including not only the head, but also on the face, arms, legs, and back. This is not a sexual type of hair (pubic hair or underarm hair), but rather an increase in the normal body hair found in all infants and children. Many parents find this increased hair growth a nuisance and many will use different methods to get rid of the hair, such as shaving, etc.

Like all drugs, Diazoxide may cause an unpredictable allergic reaction consisting of skin rashes or a more serious allergic reaction. It can also cause a decrease in the blood count. Finally, some children experience nausea, and loss of appetite.

Overall, Diazoxide is a very serious drug and when effective, it usually works extremely well.

Most children, who start on diazoxide and in whom it works very well, will be on it for many years. However the dose is not always increased as the child gets bigger. This is something that the individual physician looking after the child will decide based on blood glucose control.

Congenital HI Kids .org

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