Frequently Asked Questions for Parents of Children with PH

Group of PH childrenWhat causes pulmonary hypertension in children?

The causes of pulmonary hypertension in children are essentially the same as in adults; in some cases it is associated with another medical condition such as congenital heart disease or other lung disease; and in other cases we simply don’t know the cause (idiopathic PH). A small percentage of patients have familial pulmonary arterial hypertension, which is an inherited condition.

I’ve read about pulmonary hypertension, but most of what I read is about adults with PH. What are the primary differences between PH in adults and PH in children?

The primary difference between PH in adults and PH in children is in the treatment. Until recently, there have been very few clinical trials that study treatments specifically for children. Because of this, while the medications used for children are the same as adults, there aren’t specified pediatric doses, which makes going to a pediatric pulmonary hypertension specialist critical. Weight is a very big consideration in determining an appropriate dose for children as opposed to adults where there is standard dosing.

What kinds of treatment are available for children with PH?

There are many medications that are currently used for the treatment of PH in children. They are given in different ways: by mouth, by inhalation, by continuous intravenous infusion (directly into the vein) and continuous subcutaneous infusion (beneath the skin). Medications such as bosentan (TracleerTM) and ambrisentan (LetairisTM) are oral therapies that work on reversing effects of endothelin, a substance that PH patients tend to have too much of in their bodies. Sildenafil (RevatioTM) and tadalafil (AdcircaTM) are oral medications that help to produce more of the body’s natural vasodilators (nitric oxide). There are several medications that work by mimicking prostaglandins, which PH patients tend to have very little of in their bodies. These include epoprostenol (FlolanTM), which is administered intravenously; treprostinil (RemodulinTM and TyvasoTM), which can be administered intravenously, subcutaneously or by inhalation; and iloprost (VentavisTM), which is an inhalation therapy. It is also possible that your child will be prescribed anticoagulation therapy such as warfarin (CoumadinTM) to help prevent blood clots, diuretics to reduce fluid retention, and oxygen.

What kinds of side effects will my child experience as a result of PH medications?

The most common side effects for all PH medications include headache, nasal congestion, nausea, hypotension (low blood pressure), dizziness and flushing (feeling of warmth). TracleerTM and LetairisTM could adversely affect the liver (although this is rare), so monthly blood tests are required to monitor liver function. Inhaled therapies (TyvasoTM and VentavisTM) could also cause cough and throat irritation. Side effects of FlolanTM and RemodulinTM also include diarrhea and jaw pain and infusion site pain (subcutaneous administration only). You should discuss all side effects with your medical team even if they seem unusual or are not listed here.

What type of doctor should my son or daughter be seeing for PH?

It is important that your child be treated by a pediatric pulmonary hypertension specialist. This is usually a pediatric cardiologist or pulmonologist who has extensive training in PH and treats many children with PH. View a listing of pediatric PH specialists

How often should we see our PH specialist?

This will vary depending on your specialist, but be prepared to see your doctor every three to six months to monitor symptoms and treatment.

How will PH affect my child’s growth and development?

Most children with idiopathic pulmonary hypertension have normal growth and development; however, when it is associated with another medical condition, there could be developmental issues that may need to be addressed. For example, if breathing takes significantly more effort, their weight gain may not be normal since they use calories to breathe.

Will the PH get worse as my child gets older?

There is no known cure for pulmonary hypertension, but fortunately there are many treatments available that help prevent disease progression and support improvement in PH symptoms. Response to treatment varies from patient to patient, so communicating with your medical team will be important in determining the right treatment for your child. New research is being conducted all the time that will hopefully result in additional treatment options.

Can my child play with other kids? What about sports?

Your child can absolutely play with other children. Just be aware that your child may have to rest more frequently than other children depending on the activity and should avoid playing with children who might have the flu or upper respiratory infection, etc. What sports your child can play will depend on the type of treatment your child is on and the severity of their disease. Your medical team will be able to assist you in these kinds of decisions.

What kinds of questions should I be asking my child’s medical team?

It’s important to understand why certain tests are being done, what treatment options are available for your child, medication side effects, symptoms that warrant a call to the doctor, and what activities your child can and can’t do. You should ask how often you should see them and what to expect at each visit. It’s also important to find out how to reach your medical team after normal business hours. Keep a list of all your questions between appointments and remember that there are no silly questions, so don’t be afraid to ask!

Answers provided by Daniela Brady, RN, Pulmonary Hypertension Center at the Columbia University Medical Center

The information provided on the PHA website is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.

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The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.