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FAQ
1. What is Hemihypertrophy?
2. How do I know what kind of hemi my child has? (idiopathic, complex, cross, etc.)
3. How rare is Hemihypertrophy?
4. Will hemi ever go away?
5. Will my child live a normal life?
6. Will my child be able to participate in sports?
7. Won't everybody notice? What do I say?
8. How do I tell my child or his/her siblings what is wrong?
9. Should I try to cover it up so no one notices?
10. What is the life expectancy with hemihypertrophy?
11. Will Hemi affect me as an adult?
12. Is hemi inherited? Will my next child have it? Will his children have it?
13. Are children with hemi developmentally delayed? Miss milestones? Late walking?
14. What is the chance that hemi is the result of a bigger condition? What conditions?
15. Can Hemihypertrophy be a syndrome on its own, or is it predominately associated with other syndromes?
16. What Doctors should we see in association to the hemihypertrophy?
17. Why do I need to see genetics if my pediatrician already has diagnosed my child with Hemihypertrophy?
18. How is the hemi going to affect my child's ability to walk?
19. Will my child's hemi/LLD get better or worse?
20. What's an LLD?
21. How often should the measurements of the limbs be taken to monitor growth?
22. Should baseline x-rays be done to keep a consistent record of development? How often should they be done?
23. At what point should we consider the surgeries to address leg length discrepancies (LLD)?
24. What kind of procedures are available for the reduction of the girth and length in the affected limbs?
25. How bad should the LLD be before a lift is put in the shoe?
26. Where can I get a lift? Who would write the prescription? Won't everybody notice?
27. What types of organizations can help with the financial cost of lifts/orthotics if insurance doesn't cover them?
28. Where can we find shoes in 2 different sizes? Will anyone sell them in mismatched pairs or do we have to buy 2 separate pairs? What style shoes will work with a lift?
29. Will I need to make adjustments in my child's clothing?
30. What kind of tests should we get done to monitor the hemi? Who should be ordering these tests? How often should the tests be done?
31. The standard protocol/testing is found on the BWS website. What if my child doesn't have BWS?
32. Why are the frequent screenings necessary, can't I do them less often and be just as effective?
33. What is the risk of cancer associated with Hemi? What kinds of cancer are they?
34. Why is there an increased risk for cancer?
35. What are the symptoms of Wilm's Tumors and hepatoblastoma's?
36. What is AFP? What are the expected levels? What if this month's AFP level is higher than the last test?
37. If my family has a high history of cancer, should we continue the screening process for longer?
38. What issues can hemi cause with the internal organs?
39. Can hemi affect the pelvis/hips?
40. What is the possibility for scoliosis? How can we prevent this?
41. What kind of issues can hemi cause with the head and/or brain?
42. If there are differences in the brain size will my child have learning disabilities?
43. If there are differences in the mouth/teeth area, will my child's teeth grow in differently? Will he/she have speech problems?
44. Does Hemi cause pain How can we help make the pain easier to handle?
45. What other complications can go along with hemi?
46. What is the difference between Hemi and Edema?
47. My doctor told me to just “wait and see” if my child's LLD goes away on it's own. Can that Happen?
48. How can I get my doctors to put in referrals if they think they are unnecessary?
49. Will I have problems with my insurance company covering the cost of the frequent screening or lifts?
1. Hemihypertrophy is a greater than normal asymmetry between the right and left sides of the body.
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2. The type of hemi is determined by what the hemi is affecting and if it stands alone or as a result of a larger Syndrome. The different types of hemihypertrophy are explained on the definitions page. (non-syndromic/isolated, crossed, complex/total, or simple)
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3. Hemi is thought to occur in about 1 in 14,000 people. Isolated hemihypertrophy occurs in about one in 86,000 live births. Females and males are affected equally.
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4. No, hemi will never go away. There are times when it will appear to be more or less noticeable but it will never completely go away.
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5. Your child's life will be as normal as you make it. They will have more doctor's appointments and more things to deal with than the average child but it will be normal to them so they won't know any different. You as a parent just need to remember that they are as normal as any other child and can do just as much. Make sure you don't treat them any different.
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6. Your child should be able to participate in sports or activities just fine. The only limitations will be due to the growth of the affected limbs and the pain caused by it. If they want to try something, let them. It won't hurt and might even help with the movement limitations of the affected limbs.
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7. Other people will notice but most likely only because you said something. Unless you point it out it is very likely that people will just walk by. If they do happen to notice due to a large difference then you can tell them what you feel comfortable with. Like simply she/he has a growth condition but is perfectly healthy. Something that simple will satisfy most people.
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8. If your child is diagnosed as an infant then they will grow up with hemi and will know what is wrong with them. You may need to explain the need for the testing but, that is something you can do when you feel they are old enough to understand.
As for educating your other children. DO NOT tell them that their sibling is sick. The affected child IS NOT sick. They have a growth condition that will cause them to grow differently. If you start telling people or their siblings that they are sick then your hemi child will be treated that way and that is not what a parent wants. You want your child to be treated like any other child.
You need to judge how much you tell your other children based on their age. You don't want to tell a 4 year old everything but you don't want a 10 year old to think nothing is wrong. As a parent you need to decide what you think your child is mature enough to handle. Just use your judgment and don't overwhelm your child with to much information. Keep it as simple as possible and work more information in later.
For example: My 4 yr old knows that her brother has a growth condition and he has to go to the doctors a lot to make sure he stays healthy. He also has a birthmark and she knows that this makes him special like her Blue eyes make her special. She is OK with this explanation for now. When she gets older we will explain things in more detail but at this time her mind is to young to understand completely.
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9. Definitely not. Treat him like you would any other child. If you cover it up and act like something is wrong then he/she will grow up thinking something is wrong with them and they aren't good enough. Treat them like the little angel they are and no different than any other child.
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10. There is no shortened life expectancy from the hemi itself. The things that can affect the life expectancy are cancers and any other conditions that you may have in addition to hemihypertrophy.
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11. If you have hemi as a child then Yes it will affect you as an adult. You will still have to deal with any internal issues that were caused as a result of your hemi (kidney conditions, or other affected organs). Complications from childhood cancer and mental retardation can also cause problems. Asymmetry of the limbs can interfere with their proper function and cause pain. Insecurities due to disfigurement are possible and can be addressed through support groups or therapy.
Adults with Hemi often report back pain, hip pain, and neck pain. Since adults with Hemi
have grown up with the condition, they usually know how to manage discomfort on their
own. However, adults should see an Orthopedist or Physical Therapist if they feel their
discomfort is out of control.
Also, adults who never saw a Geneticist as a child might want to do so, since many cases
of Hemi are associated with syndromes that can cause additional problems
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12. Hemi can be inherited but it has been found that it is usually not inherited. In most cases hemi is caused by a gene mutation during pregnancy. If it is hereditary in your case then there is a possibility that your other children will be affected but no guarantee.
As for your child's children having hemi, that depends on how your child got it. If your child inherited it then there is always a chance they will pass it on. If it was a gene mutation that caused your child to have hemi then it is unknown how it will pass down to future generations.
Genetic counseling can help clarify questions families might have about passing Hemi on.
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13. Children with hemi sometimes take longer to hit their milestones. Some will need physical therapy to assist. Because of the increased bulk and length of the affected side it is harder for the child to move. They have more weight to hold up and may need a little more time for their muscles to be able to support them. If your child has any problems reaching speech or mental milestones then you may want to consider having them evaluated by their doctor to make sure something more isn't going on.
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14. Hemi can result from a bigger condition/syndrome. If your child has 2 major conditions (ex. Birthmarks, hemangiomas, deformities, hemihypertrophy, etc.) then you need to see a Geneticist to have them evaluated. The geneticist will be able to identify if the hemi is caused by a bigger issue or if it stands independently. Some of these Syndromes are Klippel-Trenaunay Syndrome, Beckwith-Wiedemann Syndrome, Proteus Syndrome, and CMTC.
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15. Hemi can most definitely stand alone. This is called Isolated Hemihypertrophy and is not associated with any other Syndromes.
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16. The Doctors you should see are based on the extensiveness of the hemi. There are very mild cases of hemi in which only an orthopedist and a geneticist are required. There are also very severe cases where all the doctor's on the treatment page are necessary plus a few more.
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17. The geneticist will be able to identify if something more is going on and will also be the one to set up the recommended screening protocol. They should let you know what other doctors you should see in connection to the hemi and take care of referrals if they are required by your insurance company.
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18. This depends on the severity of the LLD. Your child should be able to walk fine. It may be delayed as they are getting used to balancing with the length difference but they should progress fine. You might also need a shoe lift to aid in walking and sometimes a physical therapist is required. An Orthopedist will be able to determine if either one is necessary or not.
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19. This depends on the severity of the hemi that your child has. There can be a significant change in the LLD over time and there could be almost no change over time. It is hard to determine this as every case is very different and it is hard to give an accurate estimate as to how a person will grow.
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20. An LLD is a Leg Length Discrepancy. This is the difference in length between the left and the right leg when Hemi is involved.
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21. This will be determined by the individual Orthopedist. Most Doctor's recommend that we get measurements done by the pediatrician or geneticist every 6 months to keep a consistent record of growth.
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22. Definitely. X-rays should be taken at least once a year to monitor growth of the affected limbs. This way there is always a consistent record of bone and tissue development.
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23. Discrepancies of 1 cm to 1.5 cm are common among the general population, generally do not cause any symptoms, and may not require treatment. Leg lengthening or shortening involves a variety of surgical procedures used to correct legs of unequal lengths, a condition referred to as limb length discrepancy (LLD). LLD occurs because a leg bone grows more slowly in one leg than on the other leg. Surgical treatment is indicated for discrepancies exceeding 1 in (2.5 cm)
Recommended treatment for each level (0 to >20cm) of LLD (Measurements are in cm):
0 to 2: Nonsurgical treatment (Shoe lifts)
3 to 5: Shorten Longer Limb (epiphysiodesis aka growth plate arrest, or bone shortening aka resection)
6 - 14: Lengthen Shorter Limb (Distraction Osteogenesis - Bone lengthening)
15 - 19: Combining Bone Shortening and Lengthening
20 or greater: Recommend Prosthesis or amputation
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24. Leg girth is usually not treated unless it becomes a major issue causing pain or mobility problems. If this does become an issue then a Debulking Surgery (Liposuction) is the most common treatment. Before having something like this done, you need to consult with your doctor. If you or your child has a Syndrome associated with the Hemi then debulking might not be recommended, due to other complications.
The arm is almost never treated for length or girth differences. Depending on the mental issues caused by the appearance of the arm/limb, your doctor may consider treatment for the individual arm/limb in question.
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25. The LLD can be as small as ¼ of an inch when you doctor prescribes a lift. This is up to the individual orthopedist. Some won't prescribe a lift until the difference is at least ½ inch or more.
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26. Referrals or prescriptions for lifts can be ordered by the Orthopedist, Geneticist, Physical Therapist, Pediatrician, Podiatrist, and General Practitioner. Pretty much any doctor that your child sees can issue the prescription/referral for their lifts. Some people may notice the lift attached to the shoe. You only need to tell them what you feel comfortable with. Everyone will be curious at some point and the only way people learn is through education. Sometimes we need to be the educators.
**In the US you do not always need a prescription to get lifts made or ordered. If you go to an Orthotic company and meet with the Orthotist they can make the lifts for you and some places will also measure. No referral required. If you do need a referral, for insurance purposes, you can get the lift ordered through a prosthetic or Orthotic company. Your prescribing doctor should be able to lead you to the right place in your area.
**England- You need a referral to a podiatrist, who can measure for the lift and order it. This is commonly done through the hospital (NHS).
**In Kenya you can get them at any hospital (as they order from somewhere else) or you can get them from organizations for physically disabled that also make them.
. **Australia- You can get lifts and braces made by an Orthotics technician, who tailor makes them to suit. The Orthopedic surgeon can give the referral necessary.
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27. This varies from State to State and Country to Country. In America look into your local Social Services agency to see what they have to offer. Medicaid also helps offset these costs. Sometimes the local organizations will also be willing to help offset your costs. (Ex. Lions Club International, Shriner's, etc.) As for other countries, check with your insurance company and the local government to see what they have available for aid.
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28. A lot of places will sell you shoes in 2 different sizes. You need to talk to the manager and tell them your story. Sometimes they will work with you and give you a discount on the second pair of shoes. Nordstrom's will definitely sell two different sizes in one pair. They also carry a large variety of wide shoes. www.Nike.com will sell 2 different sizes but you need to call them for details. Most shoes will work with a small lift insert (¼- ½ inch). If you need a higher lift then you may want to talk to the place you are getting the lift through. They will be able to recommend a style shoe that will work best with the lift prescribed.
You might consider registering with the National Odd Shoe Exchange as they deal with only "New" shoes that people have had to purchase two pair to get a match and have donated the other mixed matched sizes. Likewise you might consider being a donor if you ever have to purchase two pair as well. Follow the resource link to their web site.
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29. The answer is…maybe. If the LLD is great enough to cause the clothes on the non-hemi side to be too long then you can simply hem that side. If the difference is in the arms, then just simply rolling up a sleeve should do the trick.
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30. Every child with hemi should be screened for kidney, liver and adrenal tumors via abdominal ultrasound and AFP blood work. The abdominal ultrasound should be done every three months until age 8. The AFP blood work should be completed every 6 weeks until age 4. Any doctor can order the tests. Your pediatrician, your orthopedist or your geneticist can all order your child’s tests.
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31. A negative BWS test does not mean that your child does not have BWS. Even if you are absolutely certain that your child does not have BWS, the same screening protocol is recommended. Even if your child’s hemihypertrophy only affects one finger, you should still follow the same screening protocol.
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32. The frequent screenings are necessary because hemihypertrophy is an overgrowth syndrome. If your child develops a malignancy in the kidney or liver, then it will likely spread rapidly. Early detection is the best defense for a full recovery.
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33. Children with hemi are more likely to develop cancers of the liver (hepatoblastoma), kidney (Wilm's Tumor), and adrenals (adrenal carcinoma). Approximately 6% of children affected by hemi develop these cancers with Wilm's tumors being the most common and hepatoblastoma's being the least common.
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34. Hemihypertrophy alters the bodies ability to control tissue and bone growth resulting in a more rapid development of cells in the affected areas. Because the cells are developing so fast there is a higher chance that they may develop abnormally resulting in Kidney, Liver and Adrenal cancers.
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35. **Wilm’s Tumors (Kidney Cancer -aka. Nephroblastoma) Common Symptoms: A mass or lump found in the abdomen of a healthy child, blood in the urine, fever, diarrhea, weight loss, abdominal pain, shortness of breath, lethargy (feeling tired and in general unwell), anemia, and high blood pressure. Wilm's tumors are the most common kind of kidney cancer in children. 8% of cancer found in children are Wilm's tumors. Wilm's tumors are slightly more common in girls, and those of African American decent. It affects children age six and under, it is highly uncommon for a child to have a Wilm’s tumor after the age of six.
**Hepatoblastoma-(Liver Cancer) Common Symptoms- Swollen abdomen or an abdominal mass, weight loss or decreased appetite, vomiting, abdominal pain, jaundice(yellowing of the skin and eyes), anemia, elevated AFP, possible back pain or shortness of breath as a result of the pressure from the tumor. Hepatoblastoma is usually found in children under the age of five, and it is extremely rare for a child over the age of five to have it. Most commonly found in children under the age of two, more common in males than females, and it is about five times more likely to be found in white children than African American children. A Hepatoblastoma tumor is from an abnormal rapid cell growth in the liver. Hepatoblastoma is rare and is only found in one child per million born.
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36. AFP stands for Alpha-fetoprotein. This protein is naturally produced by the liver, and at the same time if it is elevated, it can be detection for liver cancers. The reason as to why the liver produces this protein is unknown. AFP can only be detected in blood work. It is natural for an infant’s AFP to be slightly elevated until the age of two, when the child is growing a lot in such a short amount of time. Babies who are premature also have very elevated AFP’s. A slightly elevated AFP would be around 24-30 micrograms/ml, but can be considered normal for growing infants to the age of 2. After the age of two, a normal AFP is considered less than 10 micrograms/ml. Month to month, an AFP count can vary. The AFP can elevate if the child has recently gone though a growth spurt, or if the child is getting ready to go through a growth spurt. AFP counts become concerning if the count continues an upwards trend (EX- 8, 16, 32, 64, 128) or if the AFP suddenly reached the hundreds or thousands and should be investigated further by a physician.
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37. It is recommended that children with Hemihypertrophy get regular abdominal ultrasounds until age 8 and blood work done until age 4. After that, the recommendations are that the child has thorough physical examinations every six months until they stop growing after puberty. This can vary, depending on your child’s specific Hemi issues, and what types of cancers are prevalent in your family’s history. Only your child’s doctor can determine if further testing is necessary based on your child’s family and personal history.
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38. Issues involving internal organs will vary depending on what type of Hemihypertrophy your child has, whether it affects only one limb or the whole side of a body. It has been shown that the possibility of having one kidney, testis, ovary, or adrenal gland larger is more common with complete hemi and not always noticeable on the outside of the body. A doctor should do a full work up on a child with suspected Hemi, to see what organs, if any, may be involved. This should also be checked regularly at ultrasound visits just to monitor growth to detect any changes.
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39. Hemi itself will not affect the pelvis or hips. What will is the LLD. If the LLD is significant enough and a proper lift is not used then it can cause your hips to shift and tilt. This can cause a lot of problems in the future and as an adult with dislocation and other issues. For females this may cause problems with natural childbirth due to hip pains and a shifted pelvis (pelvic obliquity).
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40. The risk for Scoliosis is very good if the affected person does not wear the proper lifts. The spinal curvature is not considered a deformity because once the limb length is equalized it will resolve itself.
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41. If hemi affects the head, the person needs to be evaluated by a Neurosurgeon to check for hydrocephalus and hemimegalencephaly. If either one of these are found then you need to talk with your doctors about the complication they can cause with development. If neither are found then it is just a growth difference. your doctor will be able to tell you where to go from here. If the difference becomes very disfiguring then a plastic surgeon should evaluate the situation.
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42. If your child is diagnosed with hemimegalencephaly or hydrocephalus then there could possibly be learning/developmental disabilities. If it is just a mild difference between one side of the brain and the other then there will likely be no developmental issues.
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43. Hemifacial Hyperplasia is what it is called when half of your face is affected by hemi. The result from this could range from very mild to very extreme. The bony and soft tissue in the affected side will grow larger than the unaffected side. This will likely cause problems with the teeth, mouth, tongue, and jaw region. This condition does not cause mental retardation. These difference can cause speech problems due to the shape of the enlarged bones and the enlarged tongue. Speech therapy will help if this becomes an issue.
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44. Depending on how an individual’s body is affected by Hemi, there may be some pain or discomfort associated with Hemi. Since Hemi affects each individual differently, it is difficult to say if a particular individual will experience pain, or what kind of pain they will experience.
Some parents report that their children with Hemi have more “growing pains.” People with Hemi may develop knee, hip, and back pain. Some people with Hemi also report TMJ jaw pain.
If a person with Hemi experiences pain, they should see their doctor. In many cases, physical therapy can help. Pilate's, Yoga, even Belly Dancing, may also help individuals with Hemi experience less discomfort by strengthening their backs and abdominal muscles, and increasing flexibility.
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45. Aside from the increased risk of cancer and the visible overgrowth people with Hemi are prone to other issues as well. Inguinal hernias, undescended testicles, and unusual kidney's (renal cysts or horseshoe-shaped kidneys) are more common in children with hemihypertrophy whether or not they have other syndromes. The skin is often thicker on the affected side. There may be more hair on the corresponding side. Bone age is often accelerated in the involved compared to the uninvolved side.
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46. Some people confuse Hemi and Edema. Hemi is an overgrowth syndrome, which affects an individual’s bone growth. Edema is swelling caused by fluid trapped in the body’s tissues. For more information please visit the resource link.
Also, some people may confuse the symptoms of Edema and Lymphedema. Please see this resource link;
If your child suffers from Lymphedema, you may consider joining this yahoo group: childrenwithlymphedema@yahoogroups.com
People with Edema or Lymphedema should consult their doctors.
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47. Hemi does not go away. Your child should begin the recommended protocol of cancer screening as soon as Hemi is diagnosed. Your child should also see a geneticist.
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48. You need to find out what your insurance requirements are and then your make sure your doctor has the information they require to recommend the referral. Every country deals with this differently and this is something you may need to contact your healthcare system to find out how to pursue it.
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49. This is another question that you will need to contact your healthcare system to find out what they will and won't cover. Every system is different and some may cover some things whereas others won't.
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Please keep in mind that these answers are being compiled by non-medical personnel, but rather by parents, family, and friends of those who live with Hemi everyday. Most of these comments are based on facts gathered from the Internet and through medical abstracts. In the case where "No" information was located for a question, the members answers were collectively gathered and entered. They do live on the front lines with Hemi and their personal experience's speak volumes.
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