Mucopolysaccharidosis Type VI

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Where can I find pictures of other people with Mucopolysaccharidosis Type VI?

There are many support groups and societies that aim to educate the public about mucopolysaccharidosis type VI. Visit www.maroteaux-lamy.com to see some pictures of people living with mucopolysaccharidosis type VI.

References
  • www.maroteaux-lamy.com
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How long do people with Mucopolysaccharidosis Type VI usually live?

Do people with Mucopolysaccharidosis Type VI have normal intelligence?

How many people around the world have Mucopolysaccharidosis Type VI?

Are there special modifications I need to make to my house for my child with Mucopolysaccharidosis Type VI?

Are there school specific accommodations my child with Mucopolysaccharidosis Type VI may need?

Does everyone with Mucopolysaccharidosis Type VI have skeletal problems?

Does everyone with Mucopolysaccharidosis Type VI have heart problems?

Does everyone with Mucopolysaccharidosis Type VI have a large liver or spleen?

Does everyone with Mucopolysaccharidosis Type VI have hearing loss?

Does everyone with Mucopolysaccharidosis Type VI have vision loss?

Does everyone with Mucopolysaccharidosis Type VI have growth problems?

Can people with Mucopolysaccharidosis Type VI use growth hormone?

Can people with Mucopolysaccharidosis Type VI have children?

How do I know if I could have a child with Mucopolysaccharidosis Type VI?

I am currently pregnant; can I find out if my child has Mucopolysaccharidosis Type VI before they are born?

How can I educate my doctor about Mucopolysaccharidosis Type VI?

How can I find a doctor near me that has experience treating people with Mucopolysaccharidosis Type VI?

How old would a person be when they start showing signs of Mucopolysaccharidosis Type VI?

How can a stem cell transplant help with the symptoms of Mucopolysaccharidosis Type VI?

My partner and I are carriers of Mucopolysaccharidosis Type VI and already have a child with this condition. What is the chance that we would have another child with this condition?

Will my child with Mucopolysaccharidosis Type VI be able to live independently?

Do people with Mucopolysaccharidosis Type VI that use enzyme replacement therapy (ERT) live longer than those that don't?

What sort of management for skeletal problems is available for people with Mucopolysaccharidosis Type VI?

What sort of management for heart problems is available for people with Mucopolysaccharidosis Type VI?

What sort of management for eye problems is available for people with Mucopolysaccharidosis Type VI?

Are there other eye problems besides corneal clouding that people with Mucopolysaccharidosis Type VI can have?

What sort of management for hearing problems is available for people with Mucopolysaccharidosis Type VI?

What are some of the specific skeletal problems that people with Mucopolysaccharidosis Type VI can have?

What are some of the Ear-Nose-and Throat problems that people with Mucopolysaccharidosis Type VI can have?

What management is available for the lung and breathing problems that people with Mucopolysaccharidosis Type VI can have?

How can an amniocentesis tell me if my baby has Mucopolysaccharidosis Type VI?

How can a chorionic villus sampling (CVS) tell me if my baby has Mucopolysaccharidosis Type VI?

If my baby has Mucopolysaccharidosis Type VI, will this condition cause any problems for him or her before birth?

What management is available for the sleep apnea that can be caused by Mucopolysaccharidosis Type VI?

Are there any surgeries that could be helpful for people with Mucopolysaccharidosis Type VI?

I am a carrier of Mucopolysaccharidosis Type VI. How do I find out if my children could be at risk to have Mucopolysaccharidosis Type VI?

I am a carrier of Mucopolysaccharidosis Type VI. Could my children have a child with Mucopolysaccharidosis Type VI?

Are there certain jobs people with Mucopolysaccharidosis Type VI should avoid?

Are there certain sports or other activities people with Mucopolysaccharidosis Type VI should avoid?

How much does using enzyme replacement therapy (ERT) cost for Mucopolysaccharidosis Type VI?

How often do people with Mucopolysaccharidosis Type VI need to have enzyme replacement therapy (ERT)?

Can people with Mucopolysaccharidosis Type VI have enzyme replacement therapy (ERT) at their home?

How long does it take to have an enzyme replacement therapy (ERT) infusion for Mucopolysaccharidosis Type VI?

Can a bone marrow transplant help with the symptoms of Mucopolysaccharidosis Type VI?

Are problems during pregnancy common for people with Mucopolysaccharidosis Type VI?

If two people with Mucopolysaccharidosis Type VI have a child, will their child also have Mucopolysaccharidosis Type VI?

If more than one person in the same family has Mucopolysaccharidosis Type VI, will they all have similar symptoms?

Is Mucopolysaccharidosis Type VI found in other animals or just in humans?

Does the type of genetic changes a person has in the ARSB gene impact the type of symptoms they may have associated with Mucopolysaccharidosis Type VI?

How much arylsulfatase B enzyme activity is considered to be normal?

How much arylsulfatase B enzyme activity do people with Mucopolysaccharidosis Type VI usually have?

Are there other conditions where people may have high levels of dermatan sulfate in their urine besides Mucopolysaccharidosis Type VI?

Can measuring arylsulfatase B enzyme activity help determine if someone is a carrier of Mucopolysaccharidosis Type VI?

Can occupational therapy help the symptoms of Mucopolysaccharidosis Type VI?

Can physical therapy help the symptoms of Mucopolysaccharidosis Type VI?

Can speech therapy help the symptoms of Mucopolysaccharidosis Type VI?

Is weight management important for people with Mucopolysaccharidosis Type VI?

How can I manage my weight with Mucopolysaccharidosis Type VI?

Can people with Mucopolysaccharidosis Type VI have anesthesia during surgery?

What can I do if my child with Mucopolysaccharidosis Type VI has behavioral problems or signs of depression?

Why do some people with Mucopolysaccharidosis Type VI have very cold hands and feet?

Why do some people with Mucopolysaccharidosis Type VI have a hard time with blood draws?

Where can caregivers of people with Mucopolysaccharidosis Type VI get support?

Are infections something that is common in people with Mucopolysaccharidosis Type VI?

Will my child with Mucopolysaccharidosis Type VI go through puberty at the same time as their peers?

My doctor says I have "dysostosis multiplex"; what does that mean?

How long do people with Mucopolysaccharidosis Type VI usually live?

People with mucopolysaccharidosis type VI (MPS VI) have differences in how severe their disease is. Because of this, people with the slow-progressing form of MPS VI can have a reasonably normal life span. People with the more severe form in the past did not usually live past their 20s, but with newly available treatment and better management for people with MPS Type VI, it is hard to predict the exact lifespan of a person with this condition.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5#sec10
Do people with Mucopolysaccharidosis Type VI have normal intelligence?

Intelligence is typically normal for people with mucopolysaccharidosis type VI (MPS VI) and people with this condition usually succeed in school. If a person with MPS VI has less than average intelligence, this is likely because of normal variation and not because of having MPS VI.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi
How many people around the world have Mucopolysaccharidosis Type VI?

It is estimated that between 1 in every 215,000 to 250,000 people have mucopolysaccharidosis type VI, although the exact number is unknown.

References
  • http://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Are there special modifications I need to make to my house for my child with Mucopolysaccharidosis Type VI?

Many families may find that they need to make modifications to the home of a person with mucopolysaccharidosis type VI (MPS VI). Some people who are very short because of the skeletal issues from MPS VI may need to have kitchen and bathroom fixtures placed at a lower level. Other people who have to be in a wheelchair because of mobility issues may need their home modified to become more wheelchair accessible. Other changes to a person's home may depend on their individual needs and how much they want independent living.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Are there school specific accommodations my child with Mucopolysaccharidosis Type VI may need?

Although mucopolysaccharidosis type VI (MPS VI) does not affect a person's intelligence, children with this genetic condition may need specific resources in school. These may include a one-on-one school assistant, special school furniture, or individual access to a computer for assignments. It is important that your school and their staff know about the resources that your child may need so that they can make sure these resources are available.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Does everyone with Mucopolysaccharidosis Type VI have skeletal problems?

Mucopolysaccharidosis type VI (MPS VI) is a condition that affects each person differently, however, almost everyone with MPS VI has some skeletal problems that affect their height and the way that their bones form.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Does everyone with Mucopolysaccharidosis Type VI have heart problems?

Heart disease and problems with the heart as a result of breathing problems can be common in people with Mucopolysaccharidosis Type VI, but not everyone with Mucopolysaccharidosis has problems with their heart. The most common heart problems that people with Mucopolysaccharidosis Type VI have are weak heart muscle (cardiomyopathy), stiff heart muscle (endocardiofibroelastosis), and a blockage or a leakage in a heart valve.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Does everyone with Mucopolysaccharidosis Type VI have a large liver or spleen?

Most people with Mucopolysaccharidosis Type VI have an enlarged liver and spleen because of the buildup of glycosaminoglycans (GAGs) caused by their genetic changes in the ARSB gene. Even though most people with Mucopolysaccharidosis Type VI will have an enlarged liver and spleen, there may be some people with this condition who do not have this medical issue.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Does everyone with Mucopolysaccharidosis Type VI have hearing loss?

Some degree of hearing loss is common in people with Mucopolysaccharidosis Type VI, but there are people with this condition who do not have hearing loss.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Does everyone with Mucopolysaccharidosis Type VI have vision loss?

Eye problems that cause vision loss are a common problem for people with Mucopolysaccharidosis Type VI, but there are some people with this condition who will not have vision loss. The most common eye problems in Mucopolysaccharidosis Type VI are clouding of the cornea from the buildup of glycosaminoglycans (GAGs), or diseases where the main nerve in the eye, the optic nerve, is damaged (glaucoma). These eye problems can cause problems with vision or blindness.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Does everyone with Mucopolysaccharidosis Type VI have growth problems?

Because of the bone problems that people with Mucopolysaccharidosis Type VI have, most people with this condition have less than normal height for their age. People with the more severe form of Mucopolysaccharidosis Type VI may not grow taller than 3 or 4 feet, while those with a mild form of the condition may grow to 5 feet or taller.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Can people with Mucopolysaccharidosis Type VI use growth hormone?

There was a clinical trial studying the usefulness of growth hormone to increase the height of people with Mucopolysaccharidosis Type VI in the United States from 2008 to 2014. This study ended before there were any results, so there is still not much known about the use of growth hormone for people who have Mucopolysaccharidosis Type VI as of December 23, 2015.

References
  • https://clinicaltrials.gov/ct2/show/NCT00748969
Can people with Mucopolysaccharidosis Type VI have children?

Mucopolysaccharidosis Type VI does not usually affect a person's ability to get pregnant. However, given their small size, there could be some risks to a woman's health if she is severely affected by Mucopolysaccharidosis Type VI and tries to have a child. This is something that should be discussed with a person's doctor before they try to have a child, if possible.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
How do I know if I could have a child with Mucopolysaccharidosis Type VI?

People with Mucopolysaccharidosis Type VI usually get their two genetic changes in the ARSB gene that causes this condition from their parents. One of these genetic changes comes from each of their parents and this makes their parents "carriers" of Mucopolysaccharidosis Type VI. Carriers of a condition usually don't have medical problems, but can have a child with the condition that they are a carrier of. To find out if you are a carrier, you can talk to a doctor or genetic counselor about carrier testing for Mucopolysaccharidosis Type VI and other genetic conditions. If you are found to be a carrier of Mucopolysaccharidosis Type VI, you have a 25% chance to have a child with Mucopolysaccharidosis Type VI if your partner that you are having a child with is also a carrier of Mucopolysaccharidosis Type VI.

References
  • http://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi
  • http://mpssociety.org/mps/mps-vi/
I am currently pregnant; can I find out if my child has Mucopolysaccharidosis Type VI before they are born?

If you are currently pregnant and are concerned that your new baby could have Mucopolysaccharidosis Type VI, there are certain procedures a person can have during or before their pregnancy to find out if their baby has Mucopolysaccharidosis Type VI before they are actually born. Some of these include vitro fertilization (IVF) with preimplantation genetic diagnosis (PGD), chorionic villus sampling (CVS), or an amniocentesis. In general, genetic testing is only available during a pregnancy when the genetic changes have already been found in the family. A medical geneticist or a genetic counselor can help talk to you about these options and what information may be needed before you can have a procedure like one of these.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5#sec8
How can I educate my doctor about Mucopolysaccharidosis Type VI?

You can provide your doctor with education materials such as the information booklets found at http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf or http://www.ojrd.com/content/5/1/5.

References
  • http://www.ojrd.com/content/5/1/5
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
How can I find a doctor near me that has experience treating people with Mucopolysaccharidosis Type VI?

To find a doctor or center that specializes in metabolic genetic conditions or lysosomal storage disorders like Mucopolysaccharidosis Type VI in the United States, you can use the Clinic Locator Tool provided by www.maroteaux-lamy.com at http://www.maroteaux-lamy.com/en-us/pages/patient/ClinicLocator.aspx.

References
  • http://www.maroteaux-lamy.com/en-us/pages/patient/ClinicLocator.aspx
How old would a person be when they start showing signs of Mucopolysaccharidosis Type VI?

People with the more severe form of Mucopolysaccharidosis Type VI usually show signs of the condition around 2 to 3 years of age. People with the slower progressing Mucopolysaccharidosis Type VI may not have obvious signs of Mucopolysaccharidosis Type VI until their teen years or early adulthood.

References
  • http://www.ojrd.com/content/5/1/5#sec4
How can a stem cell transplant help with the symptoms of Mucopolysaccharidosis Type VI?

A hematopoietic stem cell transplant or HSCT replaces the missing arylsulfatase B enzyme in people with Mucopolysaccharidosis Type VI. This can improve some of the symptoms of the Mucopolysaccharidosis type VI like a large liver and spleen, joint stiffness, heart problems, some breathing problems, and hearing loss. A HSCT will not fix bone problems or eye problems for people with Mucopolysaccharidosis Type VI. HCST has significant risks to the person undergoing the procedure. The risks and benefits of HSCT should be discussed with your doctor.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
My partner and I are carriers of Mucopolysaccharidosis Type VI and already have a child with this condition. What is the chance that we would have another child with this condition?

If you and your partner are both carriers of Mucopolysaccharidosis Type VI, there is a 25% (1 in 4) chance for each of your children to be affected with Mucopolysaccharidosis Type VI.

References
  • http://www.omim.org/entry/253200
Will my child with Mucopolysaccharidosis Type VI be able to live independently?

People with Mucopolysaccharidosis Type VI should be encouraged to be as independent as possible as they grow up. Many of the people with this condition are able to live on their own later in life. Some teenagers with Mucopolysaccharidosis Type VI may have some restrictions on their independence because of their condition, but this varies from person to person.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Do people with Mucopolysaccharidosis Type VI that use enzyme replacement therapy (ERT) live longer than those that don't?

Treatment with enzyme replacement therapy (ERT) galsulfase can improve some of the symptoms of Mucopolysaccharidosis Type VI. However, as of December 23, 2015 there is no research about the effect of ERT on lifespan in people with this condition.

References
  • http://www.ojrd.com/content/5/1/5#sec9
What sort of management for skeletal problems is available for people with Mucopolysaccharidosis Type VI?

There are certain surgeries that can be done to try to improve the severity of joint and bone problems that people with Mucopolysaccharidosis Type VI. These surgeries can be to fuse some of the bones to prevent more damage or to get rid of some of the pressure that is put on the spinal cord. Anti-inflammatory medications like ibuprofen can also be used to reduce some of the joint pain that people with Mucopolysaccharidosis Type VI may have.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5#sec9
What sort of management for heart problems is available for people with Mucopolysaccharidosis Type VI?

Heart problems are common in people who have Mucopolysaccharidosis Type VI, but may not be present until later in life. The most common heart problems that people with Mucopolysaccharidosis Type VI have are weak heart muscle (cardiomyopathy), stiff heart muscle (endocardiofibroelastosis), and a blockage or a leakage in a heart valve. Some people may need to take medications or have surgery to help with their heart problems, while other people may need to just be monitored by their cardiologist every year or so with a test called an echocardiogram. It is helpful to be followed by a cardiologist that knows about Mucopolysaccharidosis Type VI or to give information to your doctor about the type of heart problems people with this condition can have.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5#sec4
What sort of management for eye problems is available for people with Mucopolysaccharidosis Type VI?

People with Mucopolysaccharidosis Type VI should have an eye doctor called an ophthalmologist who can check them regularly for vision and other eye problems. Corrective lenses can also help some of the vision problems associated with this condition. Some people with Mucopolysaccharidosis Type VI cannot look at bright light. For these people, wearing hats that cover the eyes or sunglasses can help. Some people with Mucopolysaccharidosis Type VI may also have medication or a corneal transplant to help improve their vision if their corneas have clouding that causes vision loss.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5#sec4
Are there other eye problems besides corneal clouding that people with Mucopolysaccharidosis Type VI can have?

In addition to corneal clouding, people with Mucopolysaccharidosis Type VI can have glaucoma, a problem where the optic nerve has damage and can cause blindness. People with this condition can also have strabismus (also known as being cross-eyed) and smaller visual fields than normal.

References
  • http://www.ojrd.com/content/5/1/5#sec4
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.omim.org/entry/253200
What sort of management for hearing problems is available for people with Mucopolysaccharidosis Type VI?

Some of the hearing problems associated with Mucopolysaccharidosis Type VI are made worse by frequent ear infections. There are medications people can take to prevent and treat these types of infections. For people with Mucopolysaccharidosis Type VI that have conductive hearing loss, there are a few types of ear tubes that can be inserted surgically to remove fluid and improve hearing. For people with this condition that have sensorineural, or nerve, hearing loss, hearing aids can be used to improve hearing.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
What are some of the specific skeletal problems that people with Mucopolysaccharidosis Type VI can have?

People with Mucopolysaccharidosis Type VI can have vertebrae in their back that are not as strong or developed as they are in other people. This can cause a curve of the spine and it can be slight or more severe depending on the person. The bones that connect the neck and the head can also be abnormal and make the neck unstable in people with Mucopolysaccharidosis Type VI. People with this condition can walk with their hips and knees flexed and may have knock-knees, also called genu valgum. Hands and feet in these individuals can be short and broad with fingers and toes bent. Almost everyone with Mucopolysaccharidosis Type VI has some joint stiffness and this can cause pain later in life.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
What are some of the Ear-Nose-and Throat problems that people with Mucopolysaccharidosis Type VI can have?

People with Mucopolysaccharidosis Type VI can have ear problems including frequent ear infections and hearing loss. They can also have problems keeping their airway open and breathing properly because of abnormal bone shapes and the buildup of storage material in the body. This causes some medical problems like runny noses, chronic sinus infections, enlarged tonsils, enlarged adenoids, and sleep apnea, or trouble breathing while sleeping.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
What management is available for the lung and breathing problems that people with Mucopolysaccharidosis Type VI can have?

People with Mucopolysaccharidosis Type VI can be evaluated by a pulmonologist for lung and other breathing problems. Vaccinations against diseases like the flu can be beneficial to prevent pneumonia and other respiratory infections. Because the airway can be difficult for enough air to pass through, people with Mucopolysaccharidosis Type VI should have their airway looked at before they have anesthesia for surgery or other procedures.

References
  • http://www.ojrd.com/content/5/1/5
How can an amniocentesis tell me if my baby has Mucopolysaccharidosis Type VI?

An amniocentesis is a procedure during pregnancy where some of the amniotic fluid is removed from around the baby and is tested. This fluid can be tested for arylsulfatase B enzyme levels to see if the baby has a problem with their enzyme levels and, therefore, could have Mucopolysaccharidosis Type VI. If there are known genetic changes for Mucopolysaccharidosis Type VI in the family, genetic testing could also be done on the amniotic fluid to see if the baby has two changes for Mucopolysaccharidosis Type VI, meaning that they have this condition. Talk to a doctor or a genetic counselor about the possibility of this testing if it is something you are interested in learning more about.

References
  • http://www.ojrd.com/content/5/1/5#sec8
How can a chorionic villus sampling (CVS) tell me if my baby has Mucopolysaccharidosis Type VI?

A chorionic villus sampling, or CVS, is a procedure during pregnancy where some of the finger-like pieces, or villi, of the placenta is removed and is tested. These villi can be tested for arylsulfatase B enzyme levels to see if the baby has a problem with their enzyme levels and, therefore, could have Mucopolysaccharidosis Type VI. If there are known genetic changes for Mucopolysaccharidosis Type VI in the family, genetic testing could also be done on these villi to see if the baby has two changes for Mucopolysaccharidosis Type VI, meaning that they have this condition. Talk to a doctor or a genetic counselor about the possibility of this testing if it is something you are interested in learning more about.

References
  • http://www.ojrd.com/content/5/1/5#sec8
If my baby has Mucopolysaccharidosis Type VI, will this condition cause any problems for him or her before birth?

Mucopolysaccharidosis Type VI affects different people differently and people with the more severe form of the condition usually start showing signs around 2 to 3 years of age. Babies with Mucopolysaccharidosis Type VI usually do not have medical problems before birth, but hydrocephalus, or fluid causing pressure in the skull, can occur, but usually in childhood.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.omim.org/entry/253200
What management is available for the sleep apnea that can be caused by Mucopolysaccharidosis Type VI?

If a person with Mucopolysaccharidosis Type VI has trouble breathing while sleeping, or sleep apnea, their doctor may want them to be monitored overnight for a sleep study. Surgical removal of the tonsils or adenoids may be recommended if they are blocking the airway. Breathing machines, such as CPAP and BiPAP machines, can be helpful in improving breathing during sleep as well. If breathing is seriously blocked, a hole into the airway from the front of the neck (also called a tracheotomy) may be recommended for a person with Mucopolysaccharidosis Type VI.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Are there any surgeries that could be helpful for people with Mucopolysaccharidosis Type VI?

A doctor may recommend certain surgeries to people with Mucopolysaccharidosis Type VI depending on the severity of the signs of their conditions. Some people with Mucopolysaccharidosis Type VI have surgeries to lessen the pressure on the spinal cord, to fuse the bones in the neck, to remove the tonsils or adenoids in the throat, to fix problems with the heart, and for other reasons. Each surgery a person with Mucopolysaccharidosis Type VI has should be discussed with a doctor who is knowledgeable about their condition before they have a procedure.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5#sec5
I am a carrier of Mucopolysaccharidosis Type VI. How do I find out if my children could be at risk to have Mucopolysaccharidosis Type VI?

If you are a carrier of Mucopolysaccharidosis Type VI, your future children will each have a 25% chance to have Mucopolysaccharidosis Type VI if your partner is also a carrier of the condition. You partner can have testing to determine if they are a carrier of Mucopolysaccharidosis Type VI and, therefore, if your children together could have this condition.

References
  • http://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi
  • http://www.omim.org/entry/253200
I am a carrier of Mucopolysaccharidosis Type VI. Could my children have a child with Mucopolysaccharidosis Type VI?

If you are a carrier of Mucopolysaccharidosis Type VI and your partner is not, your children would each have a 50% chance to be a carrier of this condition just like you. If one of your children is a carrier and so is their partner that they have children with, each of their children will have a 25% chance to have Mucopolysaccharidosis Type VI.

References
  • http://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi
  • http://www.omim.org/entry/253200
Are there certain jobs people with Mucopolysaccharidosis Type VI should avoid?

The physical disabilities of people with Mucopolysaccharidosis Type VI are variable and may make certain jobs more physically challenging than others. However, the Americans with Disabilities Act gives assistance to employers and employees so that people with Mucopolysaccharidosis Type VI are not prevented from having jobs in which they are successful.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Are there certain sports or other activities people with Mucopolysaccharidosis Type VI should avoid?

The physical restrictions of people with Mucopolysaccharidosis Type VI are unique to each person's symptoms and severity of disease. As of December 23, 2015 there are no general restrictions on activity that are suggested for individuals with Mucopolysaccharidosis Type VI. Doctors may encourage people with Mucopolysaccharidosis Type VI to avoid contact sports if they have an enlarged spleen or certain bone problems, but these are not general recommendations.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5
How much does using enzyme replacement therapy (ERT) cost for Mucopolysaccharidosis Type VI?

The yearly cost of using galsulfase enzyme replacement therapy (ERT) for each person with Mucopolysaccharidosis Type VI in the United States is about $441,000 as of July 16th, 2013. This is the estimated cost if a person did not have insurance, but depending on the health insurance plan that a person with Mucopolysaccharidosis Type VI has, the cost may be much less.

References
  • http://www.tandfonline.com/doi/pdf/10.1517/21678707.2013.819289
How often do people with Mucopolysaccharidosis Type VI need to have enzyme replacement therapy (ERT)?

The current recommendation of galsulfase for people with Mucopolysaccharidosis Type VI as of December 23, 2015 is one infusion every week.

References
  • http://www.naglazyme.com/about-naglazyme/
Can people with Mucopolysaccharidosis Type VI have enzyme replacement therapy (ERT) at their home?

Usually when a person with Mucopolysaccharidosis Type VI has had enzyme replacement therapy (ERT) for a significant period of time with no sign of allergic reaction or other side effects, they may be able to arrange ERT infusions at their home. The ability to have ERT infusions may depend on the recommendation of a person's doctor or the specifics of the person's insurance plan.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5
  • http://www.naglazyme.com/taking-naglazyme/home-infusion/
How long does it take to have an enzyme replacement therapy (ERT) infusion for Mucopolysaccharidosis Type VI?

The amount of time needed for a person with Mucopolysaccharidosis Type VI to have an infusion for enzyme replacement therapy (ERT) can vary from person to person. This is because the amount of ERT that each person gets is based on their body weight. Also, the first few times someone has ERT infusions, they may take much longer than usual to make sure the person does not have an allergic reaction or other side effects to the ERT drug. For this reason, having an ERT infusion could take 4 hours or more each week.

References
  • http://naglazyme.com/taking-naglazyme/treatment-tips/
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Can a bone marrow transplant help with the symptoms of Mucopolysaccharidosis Type VI?

A bone marrow transplant is a type of hematopoietic stem cell transplant, or HSCT. A HSCT replaces the missing arylsulfatase B enzyme in people with Mucopolysaccharidosis Type VI. This can improve some of the symptoms of the Mucopolysaccharidosis type VI like a large liver and spleen, joint stiffness, heart problems, some breathing problems, and hearing loss. A bone marrow transplant will not fix the bone or eye problems for people with Mucopolysaccharidosis Type VI. There are also significant risks associated with having HCST. The risks and benefits of HCST should be discussed with a healthcare provider to determine what is best for the patient.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Are problems during pregnancy common for people with Mucopolysaccharidosis Type VI?

Fertility, or the ability to become pregnant, is not affected in people with Mucopolysaccharidosis Type VI. However, there can be some medical problems that women with Mucopolysaccharidosis Type VI may experience during pregnancy due to their small size, skeletal findings, and heart issues. These medical problems vary from woman to woman with Mucopolysaccharidosis Type VI and are more common in women with the severe form of the condition that have more physical restrictions due to skeletal problems. If you are a woman with Mucopolysaccharidosis Type VI and are thinking about becoming pregnant, talk with your doctor to talk about concerns about your health during pregnancy.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
  • http://www.ojrd.com/content/5/1/5
If two people with Mucopolysaccharidosis Type VI have a child, will their child also have Mucopolysaccharidosis Type VI?

If two people with Mucopolysaccharidosis Type VI have a child, all of their children will also have Mucopolysaccharidosis Type VI. This is because the parents do not have genetic instructions for the working arylsulfatase B enzyme for their children to inherit.

References
  • http://ghr.nlm.nih.gov/condition/mucopolysaccharidosis-type-vi
If more than one person in the same family has Mucopolysaccharidosis Type VI, will they all have similar symptoms?

Even if more than one person in the same family has Mucopolysaccharidosis Type VI and the same genetic changes that causes the condition, that does not necessarily mean that they will all have the same symptoms or severity of the condition.

References
  • http://www.ojrd.com/content/5/1/5
Is Mucopolysaccharidosis Type VI found in other animals or just in humans?

There are some animals, such as cats and dogs, that can also have a condition like Mucopolysaccharidosis Type VI that is caused by low levels of arylsulfatase B enzyme.

References
  • http://www.ojrd.com/content/5/1/5
  • http://www.ncbi.nlm.nih.gov/pubmed/12892300
  • https://www.vgl.ucdavis.edu/services/cat/MPSVI.php
  • http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3401909/
Does the type of genetic changes a person has in the ARSB gene impact the type of symptoms they may have associated with Mucopolysaccharidosis Type VI?

There are some specific genetic changes that are associated with the more severe form of Mucopolysaccharidosis Type VI, but other genetic changes have been found in, both people with the severe form of Mucopolysaccharidosis Type VI and people with the mild form of Mucopolysaccharidosis Type VI. Because of this, it is hard to know what form of the condition or what symptoms of Mucopolysaccharidosis Type VI a person might have by just looking at their genetic changes. If you have a genetic testing result for the ARSB gene and want to know more information about what those changes mean, talk to a genetic counselor.

References
  • http://www.ojrd.com/content/5/1/5
How much arylsulfatase B enzyme activity is considered to be normal?

Normal levels of arylsulfatase B in the body is determined by an accepted range at certain laboratories that perform this testing. The number for the normal "cutoff" may be different at different labs because of this and can also be different depending on what type of tissue is used for the testing (white blood cells, cultured skin cells, etc.), but should be listed on the testing result no matter what type of sample was used For instance the Mayo Clinic Mayo Medical Laboratories quotes the normal range to be anything more than 6.08 nanomoles per milligram of protein in cultured skin cells. Regardless, arylsulfatase B testing would tell if a person's enzyme levels are below the normal range enough to be concerning for Mucopolysaccharidosis Type VI.

References
  • http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/8151
  • http://www.ojrd.com/content/5/1/5
How much arylsulfatase B enzyme activity do people with Mucopolysaccharidosis Type VI usually have?

People who are diagnosed with Mucopolysaccharidosis Type VI usually have less than 10% of the normal amount of arylsulfatase B enzyme when compared to people who do not have this condition.

References
  • http://www.ojrd.com/content/5/1/5
  • http://www.omim.org/entry/253200
Are there other conditions where people may have high levels of dermatan sulfate in their urine besides Mucopolysaccharidosis Type VI?

People with Hunter Syndrome, or Mucopolysaccharidosis Type II, have increased amounts dermatan sulfate in their urine but also have high levels of heparan sulfate present in their urine. High levels of dermatan sulfate points to a person having Mucopolysaccharidosis Type VI if dermatan sulfate is the only increased sulfate found in the urine.

References
  • http://www.omim.org/entry/309900
  • http://www.ncbi.nlm.nih.gov/pubmed/25345092
Can measuring arylsulfatase B enzyme activity help determine if someone is a carrier of Mucopolysaccharidosis Type VI?

Determining if someone is a carrier of Mucopolysaccharidosis Type VI by looking at their arylsulfatase B enzyme levels in the blood or in skin cells is possible. However, another form of carrier testing for Mucopolysaccharidosis Type VI is done by genetic testing that looks for changes in a person's ARSB genes.

References
  • http://www.ncbi.nlm.nih.gov/pubmed/806052
  • http://www.omim.org/entry/253200
Can occupational therapy help the symptoms of Mucopolysaccharidosis Type VI?

Occupational therapy may not directly improve the symptoms of Mucopolysaccharidosis Type VI, however, it can teach people with this condition how to use adaptive techniques so that they can be successful in home and work settings and be as independent. This type of therapy can be very useful for people with the severe form of Mucopolysaccharidosis Type VI, as they may have more physical limitations caused by bone problems or shorter height than others.

References
  • https://rarediseases.org/rare-diseases/maroteaux-lamy-syndrome/
Can physical therapy help the symptoms of Mucopolysaccharidosis Type VI?

Physical therapy can help with some of the joint pain and stiffness that people with Mucopolysaccharidosis Type VI can have. A physical therapist can also help a person with Mucopolysaccharidosis Type VI be as active as possible without causing joint pain so they can improve their joint function and overall health.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Can speech therapy help the symptoms of Mucopolysaccharidosis Type VI?

Problems with speech is not something that is common in people with Mucopolysaccharidosis Type VI. However, if you or someone you know with Mucopolysaccharidosis Type VI does not speak, has trouble speaking, or has trouble pronouncing words correctly, speech therapy can be helpful.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Is weight management important for people with Mucopolysaccharidosis Type VI?

Weight management is an important part of health for everyone, but can be especially important for the health of people with Mucopolysaccharidosis Type VI. This is because the bone problems that people with Mucopolysaccharidosis Type VI sometimes have can be made worse if they are overweight.

References
  • http://www.ojrd.com/content/5/1/5
How can I manage my weight with Mucopolysaccharidosis Type VI?

Diet and exercise are good strategies for weight management in people with Mucopolysaccharidosis Type VI. As of December 26, 2015, there are no specific diets recommended for people with Mucopolysaccharidosis Type VI. People with Mucopolysaccharidosis Type VI should exercise for general health, but should not do activities that cause pain. Physical therapists, nutritionists, and other doctors can help you with a personalized plan for weight management if it is something that you are interested in learning more about.

References
  • http://www.ojrd.com/content/5/1/5
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Can people with Mucopolysaccharidosis Type VI have anesthesia during surgery?

People with Mucopolysaccharidosis Type VI can have anesthesia during surgery, but may need special care of their airway during surgery. Since people with Mucopolysaccharidosis Type VI can have trouble breathing, extra care should be taken by an anesthesiologist and pulmonologist to find out if anesthesia could cause any problems during the surgery. There are some tests that these doctors can do to find out if the use of anesthesia during a surgery will have extra risks for a person with Mucopolysaccharidosis Type VI. If possible, use a pediatric anesthesiologist or anesthesiologist who has experience treating people with Mucopolysaccharidoses. If that is not possible, let your surgeon, anesthesiologist, and other doctors know that breathing problems can occur in people with this condition so they can be educated about this possibility.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
What can I do if my child with Mucopolysaccharidosis Type VI has behavioral problems or signs of depression?

If your child with Mucopolysaccharidosis Type VI is exhibiting behavioral problems, signs of depression, or other signs of a mental illness that may or may not be related to having a long term illness, there are a few different resources that may be helpful for them. For behavioral problems, counseling or behavioral therapy may be useful. For depression or other mental illnesses, counseling by a psychologist or being treated by a psychiatrist may be helpful for them as well.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Why do some people with Mucopolysaccharidosis Type VI have very cold hands and feet?

Some people with Mucopolysaccharidosis Type VI may have problems breathing or problems with their heart that stops their blood from circulating to the hands and feet correctly. If this happens, a person can have cold hands and feet that are not warmed as much as other parts of the body. Some people use gloves and socks to warm up their hands and feet, but other people may need to see a doctor to talk about what is causing this and how to treat it. If a person with Mucopolysaccharidosis Type VI's hands or feet turn blue one time or on a regular basis, this is something that needs to be talked about with a doctor and could mean there is a more serious problem with the heart or lungs.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Why do some people with Mucopolysaccharidosis Type VI have a hard time with blood draws?

People with Mucopolysaccharidosis Type VI can have tough and thick skin and this can make blood draws or other procedures needing an intravenous line more challenging.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Where can caregivers of people with Mucopolysaccharidosis Type VI get support?

Caregivers of people with any long term condition can be tiring and challenging and this is no different for parents or other caregivers of people with mucopolysaccharidosis type VI. If you are a caregiver in need of support, try to take time for yourself, set aside time for personal counseling, or look for other resources at http://caregiveraction.org/.

References
  • http://caregiveraction.org/
Are infections something that is common in people with Mucopolysaccharidosis Type VI?

Recurring ear and sinus infections are common in people with Mucopolysaccharidosis Type VI. Because of the problems with the airway that some people with this condition can have, respiratory infections can also be common. For this reason, flu and other vaccines that protect against these types of infections should be considered for people with Mucopolysaccharidosis Type VI.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
Will my child with Mucopolysaccharidosis Type VI go through puberty at the same time as their peers?

Teenagers with Mucopolysaccharidosis Type VI go through the normal stages of puberty but they may go through puberty later than other teenagers.

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf
My doctor says I have "dysostosis multiplex"; what does that mean?

Dysostosis multiplex is a medical term for a pattern of bone problems that are common in people with Mucopolysaccharidoses, including Mucopolysaccharidosis Type VI. These bone problems include abnormally shaped back bones (vertebrae) and ribs (spatulate), enlarged skull, too small and too thick leg bones (hypoplastic epiphyses and thickened diaphyses) and bullet-shaped finger and toe bones (metacarpals).

References
  • http://mpssociety.org/wp-content/uploads/2011/07/booklet_MPS_VI_v5.pdf

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