sickle cell disorders in Al hassa

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Common Systemic Diseases/Disordes of Dental Interest in AL Hassa Province,K.S.A. Dr. Usama M. Madany Consultant Periodontist, AL Hassa Dental center

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I-Hemolytic Anemias: > 30% (Ministry of health report 2008) II- Diabetes Mellitus: > 23.7% (Al Nozha et al 2004) III- Hypertension: 15%-18.7% (Al Hazimi et al 1998, EMRO bulletin 2001) IV- Renal failure: 12%-39% (Al Wakeel et al 2009) V- Cardiovascular Diseases:24%-36% (Al Wakeel et al 2009) VI- Bronchial Asthma: 11.7% (Al Shairi A. and Al Dawood K, 1999) Common diseases include:

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Hemolytic Anemias Sickle cell disease Sickle cell anemia (homozygous): Sickle cell trait (heterozygous) Thalassemia Alpha thalssemia Beta thalssemia Thalsssemia major (homozygous thalassemia, Cooley’s anemia) Thalssemia minor (heterozygous thalassemia, thlassemia trait) Glucose 6 Phosphate Dehydrogenase Deficiency (G6PDD, Favism): 37% (Al Ali A. 1996, Nasurullah Z et al. 2003)

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Sickle cell anemia is an inherited blood disorder caused by structurally abnormal haemoglobin (Hb S ) that causes a rigid distorted red blood cell (sickle cell) Acute crises may occur spontaneously or precipitated by Infection Dehydration Hypoxia Sedatives and local anaesthetics Cold Hemolytic Anemia Weakness Splenomegaly Heart failure Increased viscosity and clumping of RBCs Thrombosis Ischemia Infarctions

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Dental Aspects Oral Manifestations -Pale and yellow oral mucosa -Maxillary overgrowth Delayed eruption -Enamel hypoplasia- Radiographic findings

Hair on End appearance : 

Hair on End appearance

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Susceptibility to infection Bleeding tendency Susceptibility to develop crisis Problems

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Dental considerations and management Never Anesthetize or operate on patients during crisis (except for the relief of pain and treatment of infection). Use tourniquet Use GA in the clinic (GA must be carried out in a hospital with full anesthesia facilities and in close cooperation with a hematologist) Prescribe drugs that can cause respiratory depression and hypoxia such as sedatives.

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Expect Cerebral or cardiac hypoxia with minor procedure. Poor wound healing after minor surgery or extraction. Pulpal symptoms are common without obvious dental disease. Infarction of bone may result in osteomyelitis esp. in the mandible.

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Remember -Patient’s physician should be consulted about the patients myocardial status. -Preventive dental care is important. -Dental infections should be treated vigorously, if facial cellulitis develops patient should be admitted to the hospital. -Pain medications for patients with sickle cell disease include paracetamol, paracetamol with Codeine, NSAID’s and narcotics especially morphine.

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-Due to high risk of infection in such patients, prophylactic antibiotic regimen for prevention of endocarditis should be followed before any dental procedures that can cause transient bacteremia. -After surgical procedures, antibiotics ( 500 mg penicillin VK+ 4 times/day or erythromycin, 250 mg 4 times/day, for penicillin-allergic patients) should be continued for 7-10 days postoperatively. -Use of vasoconstrictor in local anesthesia is controversial, however, for short routine procedures lacking discomfort , the vasoconstrictor should not be used. If the procedure requires long profound anesthesia, 2% lidocaine with 1/100,000 adrenaline is the anesthetic of choice.

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If general anesthesia is required, it is advisable to carry out comprehensive dental treatment, including any extraction or restoration that may be required, so as to avoid a second procedure. The orthodontist should pay attention to the possible pulpal necrosis involving healthy teeth, the changes in the bone turnover during orthodontic movements, the mandibular painful episodes, and the greater susceptibility to infections. Orthodontic planning should be adjusted to restore the regional microcirculation by increasing the rest intervals as well as to reduce the movements of the teeth and the forces applied on them.

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Trait -60% of hemoglobin is HbS -Patient is asymptomatic and lives normal life -Sickle cell crisis can be precipitated by reduced oxygen tension -Few problems in management *General anesthesia better be performed in hospital with full oxygen supply *Acute infections should be treated immediately *Prophylactic antibiotic cover before surgery

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Thalassemia Group of congenital disorders characterized by deficient synthesis of either α or β chains of globin. Alpha thalssemia Beta thalassemia Imbalance in globin chain precipitation of these chains in RBCs ineffective erythropoiesis + hemolysis

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Beta thalassemia 1-Homozygous thalassemia (Cooley’s anemia) no or few normal beta chains 2-Heterozygus thalassemia Cooley’s anemia Oral manifestations Pale or/and oral mucosa Painful swelling of salivary glands Painful tongue Teeth discoloration Hyperplasia of bone marrow Large head and mongoloid characters Enlargement of premaxilla Spacing of upper ant. teeth Delayed sinuses’ pneumatization

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b a c

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Dental implications 1- Expect poor wound healing 2- Antibiotic is required to control infection without delay 3- Avoid GA in dental clinic . 3- Avoid excessive bleeding during surgery.

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Glucose 6 Phosphate Dehydrogenase Deficiency (G6PDD) Erythrocyte metabolic defect characterized by hemolysis due to denaturing the hemoglobin in presence of oxidant compounds. Life span of RBCs is 2/3 the normal and this is compensated by liver and bone marrow Diabetes Infection Oxidants compounds General anesthesia Dental management 1- Avoid oxidant drugs eg asprin, phenacetin, sulphonamides, chloramphenicol, and vit .K 2-Infection should be treated without delay Hemolysis

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Recommendations Any patient under 15 years old with a family history of hemoglopinopathies or G6PDD should be screened for hemolytic anemias before starting dental treatment.

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