Several People have asked me to explain the issues that Porter (my youngest son) has.
Porter Has:
Hemophagocytic Lymphohistiocytosis -A rare, cancer-like disorder in which both histiocytes and lymphocytes start to proliferate and attack body tissues or organs. Treatment is by chemotherapy, and in some (Porter’s) cases bone-marrow transplantation.
Bone marrow necrosis (BMN)-Bone marrow is dyeing...will need a bone marrow transplant soon.
Pernicious Anemia- Pernicious anemia is due to an inability to absorb vitamin B-12 (also known as cobalamin or Cbl) from the gastrointestinal tract.
B-cell Dysfunction (compromised immune system…it’s broken). Gets very, very sick easily and has to be hospitalized and given IV’s/Meds.
Hypohidrotic Ectodermal Dysplasia - Most people with hypohidrotic ectodermal dysplasia have a reduced ability to sweat (hypohidrosis) because they have fewer sweat glands than normal or their sweat glands do not function properly. Sweating is a major way that the body controls its temperature; as sweat evaporates from the skin, it cools the body. An inability to sweat can lead to a dangerously high body temperature (hyperthermia), particularly in hot weather. In some (Porter’s) cases, hyperthermia can cause life-threatening medical problems. Porter dehydrates quickly.
Autism-Autism is a devastating neurological and biological disorder that typically affects children between the ages in 18 months to five years of age. Autism currently affects 1 in every 91 children today. It is estimated there are over 1 million people in the United States alone with autism. Autism affects each individual differently and at different levels of severity. Some people with autism are severely affected, cannot speak, require constant one-on-one care, and are never able to live independently. While others who have less severe symptoms, can communicate, and eventually acquire the necessary skills to live on their own.
Chronic Diarrhea -for over 5 years now.
His Treatments:
Intravenous immunoglobulin (IVIG)- is a blood product administered intravenously. It contains the pooled IgG (immunoglobulin (antibody) G) extracted from the plasma of over one thousand blood donors. IVIG's effects last between 2 weeks and 3 months. IVIG is given as a plasma protein replacement therapy (IgG) for immune deficient patients who have decreased or abolished antibody production capabilities. In these immune deficient patients, IVIG is administered to maintain adequate antibody levels to prevent infections and confers a passive immunity. Treatment is given every 3–4 weeks. In the case of patients with autoimmune disease, IVIG is administered at a high dose (generally 1-2 grams IVIG per kg body weight) to attempt to decrease the severity of the autoimmune diseases.
Central line (4 years now)- is a catheter placed into a large vein in the neck (internal jugular vein or external jugular vein), chest (subclavian vein) or groin (femoral vein). It is used to administer medication or fluids, obtain blood tests (specifically the "mixed venous oxygen saturation"), and directly obtain cardiovascular measurements such as the central venous pressure.
TPN- for 12 hour nightly feeds via two pumps/central line. See link: http://www.merckmanuals.com/professional/sec01/ch003/ch003c.html
Drinks EleCare – 24-28 oz. daily for nutrition. EleCare is an amino-acid-based, hypoallergenic formula that is specifically designed for infants and children with special feeding issues.
Meds-Takes a plethora
Attends Blood Cancer Clinic- for blood draws to check platelet count, liver, pancreas, bone marrow, etc…etc…
BMT- Eventually, Porter will need a Bone Marrow Transplant to survive.
Has had several surgeries: NG tube, colonoscopy, endoscopy, enteroscopy, central line (2 x’s), will need a G tube and a Port placement soon to access blood.
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