OurStory

Treatment

Treatment of the symptoms occurring in pediatric mastocytosis consists of the prevention of exposure to agents that are known to cause symptoms in that child, and the use of medications if necessary. “Management” is at least as important as “medication”.

When symptoms are present in children with mastocytosis, it is often beneficial to treat them with medications at the time of symptoms, or with routine doses of medication if symptoms are frequent.

H1 antihistamines, such as are normally associated with relief of allergic symptoms, are the first treatment used, and can relieve most of the symptoms present in pediatric mastocytosis. It may be necessary to try several different H1 antihistamines, using each for several weeks before evaluating its effect.

H2 antihistamines, such as Pepcid (famotidine) and Zantac (ranitidine) may relieve skin symptoms that continue in spite of adequate treatment with H1 antihistamine. They also relieve symptoms from excessive stomach acid or gastric reflux.

If a child is taking both H1 and H2 antihistamines in adequate doses and still experiences frequent symptoms, there are several other medications used, depending on the symptoms.

Cromolyn (disodium cromoglycate) reduces mast cell activity. It can be used orally for GI symptoms, by inhalation for respiratory symptoms, as a nasal spray to reduce congestion, as eye drops to relieve itching and swelling of eyes, and as a topical cream to relieve skin symptoms. Cromolyn is absorbed into the system through both oral and inhalational use and, although absorption is minimal, children often experience a reduction in many of their symptoms.

Children with severe and symptomatic skin involvement (such as those with extensive urticaria pigmentosa and those with diffuse cutaneous mastocytosis) may be helped with light treatments in conjunction with taking a medication called psoralen. This treatment is referred to as PUVA, and reduces substantially the number of mast cells in the skin.

Leukotriene receptor blocking agent montelukast (Singulair) may be helpful when respiratory symptoms remain in spite of all of the above treatment, especially in children who have asthma along with their mastocytosis.

Other drugs are available for various symptoms and may be tried in consultation with a mastocytosis-experienced specialist. Aspirin and other non-steroidal anti-inflammatory drugs can cause sudden onset of severe symptoms in people with mastocytosis and are especially contraindicated in children with diffuse cutaneous mastocytosis because of their ability to cause gastrointestinal bleeding.

The emergency use of epinephrine (adrenaline) injection is controversial in very small children, for whom the available single-dose injectors would result in overdose. In children whose body weight is adequate for the use of EpiPen Jr., having one on hand and instructing the parent or caregiver in its use is an important safeguard if severe symptoms, such as loss of consciousness and shock, occur. These severe symptoms are possible in all forms of pediatric mastocytosis, and consultation with an experienced physician as to the use of EpiPen should be undertaken as soon as a child is diagnosed with mastocytosis.

Conclusion:

A diagnosis of pediatric mastocytosis may present challenges for the child and the family involved. Children with “spots” need to be reassured about social contacts, and children who experience obvious symptoms need the protection of having the adults around them educated in how to manage and treat these symptoms. Organizations such as Mastokids.Org (www.mastokids.org) can help families and affected children select useful techniques for coping with the disease while helping the child live as normal a life as possible.