The Power of Your Choice
Your choice of the IHTC Pharmacy Program directly supports your IHTC team and patient services, and activities provided to the hemophilia community. The IHTC Pharmacy Program provides savings to you and your health insurance plan.
Every patient has the right to choose their clotting factor pharmacy provider. The IHTC supports your right of choice and will assist you in making an informed decision.
Sickle cell hemoglobinopathies have the potential to cause ophthalmic complications which can affect vision. Ophthalmoscopic evaluation for these patients must include thorough examination of the posterior and peripheral retina through a dilated pupil.
Proliferative sickle retinopathy (PRS) can lead to viterous hemorrhage and retinal detachment. Laser photocoagulation is often helpful for many patients with this complication. Retinal detachment and loss of vision can be avoided in some patients who develop retinal holes or traction by early detection and treatment. Once detachment or significant vitreous hemorrhage is present, surgical intervention is necessary in an attempt to restore vision.
Outcomes may be influenced by proper gentle debridement, control of local edema, treatment of superinfection, identification and correction of nutritional deficiencies, and prevention of significant anemia.
Correction of Anemia
Transfusion therapy is usually extremely helpful in the healing course of leg ulcers. Most patients will tolerate a simple transfusion every 4 weeks. The pre-transfusion Hb should be less than 10, and the goal should be to maintain a pre-transfusion Hb S of < 30%. A transfusion course of at least six months is usually needed. Extended antigen matching and leukodepletion should be done to minimize the risk of alloimmunization. Iron load status should be monitored with serial ferritin levels.
Saline wet-to-dry dressings two to three times a day can debride adherent eschar. If this is very painful, analgesic premedication should be administered 30 minutes prior to the dressing change. EMLA cream may also be applied directly onto the wound and covered with plastic wrap for 60 minutes prior to gentle manual wound debridement.
Crystalline sodium chloride-impregnated gauze (Mesalt of Sanulla, Inc.) can be useful if copious exudate interferes with drying. Debridement using hydrocolloid dressings (DuoDerm, Convatec) can be used to liquify all dead tissue and is changed only every 4-7 days. The dressing is applied with ample margins so that the seal remains intact. It must be changed immediately if the seal is lost or if there is leakage. If the wound is deep, it can be filled with granules and then covered with the hydrocolloid dressing. Due to the liquidation and removal of devitalized tissue, the wound can appear larger when the dressing is changed. It may also have a foul odor. Once a clean bed is obtained, compression dressings can be initiated with an Unna’s boot (zinc oxide impregnated gel boots) even over a hydocolloid dressing.
Once some healing has begun, a moist dressing may be more helpful. For example, applying antibiotic ointment to the wound, covered by a petroleum gauze dressing followed by a plain gauze and elastic compression bandage wrap. Bed rest, leg elevation, and compression stockings or wraps (Coban, ACE wraps, TED hose) help control local edema.
Secondary infection with Staphyloccus aureus and Pseudomonas aeruginosa are common. Topical antibiotics in ointment or spray form (like mixtures of bacitracin, neomycin, and polymyxin B) can be helpful. Some have had success using polymyxin B powder (by itself or mixed with Dermagran solution) directly on the wound followed by a layer of collagenase ointmant. Systemic antibiotic therapy is not helpful unless the patient develops cellulitis. If cellulitis develops, or an underlying osteomyelitis is suspected, oral or parenteral antibiotics covering Staph. aureus and Pseudomonas should be initiated.
Regranex gel applied directly to the wound after gentle cleansing and debridement 1-3 times per day has been effective for some. This synthetic matrix of a tripeptide bound to a hyaluronate supports and stimulates migration of cells, granulation, and keratinocyte layer formation.
Topical applications of Granulocyte-Macrophage Colony Stimulating Factor (GM-CSF) have not proven to be effective, but subcutaneous injection of the GM-CSF directly into the ulcer has been shown to promote wound healing. The wound can be lathered with topical EMLA cream.