MANAGEMENT OF MEDICALLY

COMPROMISED DENTAL

PATIENT: ANEMIA, SICKLE

CELL DISEASE, THALASSEMIA,

G6PD

Abdul Rahman, Abrar, Ahmad BadruddinKulliyyah of DentistryInternational Islamic University Malaysia

ANEMIA: DEFINITIONReduction in the oxygen-carrying capacity of

the blood, usually associated with a decreased number of circulating RBCs or an abnormality in the Hb contained within the RBCs.

Anemia is not a disease but rather a symptom complex that may result from one of three underlying causes: (1) decreased production of RBCs (iron deficiency, pernicious anemia, folate deficiency), (2) blood loss, or (3) increased rate of destruction of circulating RBCs (hypersplenism,autoimmune destruction).

A man considered of having anemia when the Hb value below 13g/dl,While for a woman when the Hb value below 12g/dl.

TYPES OF ANEMIA

SICKLE CELL ANEMIA The RBC in sickle cell anemia becomes

sickle shaped when blood experiences lowered oxygen tension or decreased pH, or when the patient becomes dehydrated.

In patients with sickle cell anemia, more than 80% of the Hb is HbS (sickle haemoglobin). Clinical signs and symptoms of sickle cell anemia are the result of chronic anemia and small blood vessel occlusion, such as jaundice, pallor, dactylitis (hand and foot warmth and tenderness), leg ulcers, organomegaly, cardiac failure, stroke, and delays in growth development.

G-6-PD DEFICIENCY glucose-6-phosphate dehydrogenase (G-6-PD)

is an enzyme that helps the RBC to turn carbohydrates into energy.

Clinical features of G-6-PD deficiency involve acute intravascular hemolysis, which may be severe. Jaundice, palpitations, dyspnoea, and dizziness may result.

Of more than 40 drugs that can induce haemolysis, those having dental significance include acetylsalicylic acid (aspirin), phenacetin, ascorbic acid, and vitamin K.

Broad bean ingestion is the most common dietary cause of haemolytic anemia in person with G-6-PD deficiency.

THALASSEMIA Thalassemia is among the most

common genetic disorder in human. More than 4000 thalassemia patient are

registered in Malaysia in 2009. Thalassemias are classified according to

which chain of the hemoglobin molecule is affected. In α-thalassemias, production of the α-globin chain is affected, while in β-thalassemia production of the β-globin chain is affected.

ALPHA THALASSEMIA

a. Hydrops fetalis - if all 4 genes deleted.

b. Hb-H - if 3 genes deleted.

c. Mild hypochromic microcytic anemia - if 2

genes deleted (α thalassemia trait) and

usually mistaken with iron deficiency anemia.

d. Minimal effect if only 1 gene deleted. Called

as silent carrier.

BETA THALASSEMIA MINOR Thalassemia minor is not always actively

treated. While many of those with minor status do not require blood transfusion therapy they still present at risk of iron overload, particularly in the liver.

Increased gastrointestinal iron absorption is seen in all grades of beta thalassemia, and increased red blood cell destruction by the spleen due to ineffective erythropoiesis further releases additional iron into the bloodstream.

A serum ferritin test should be done to check their iron levels and guide them to further treatment if necessary.

BETA THALASSEMIA MAJOR Occurs when both  alleles have

thalassemia mutations. Can lead to severe microcytic, hypochromic anemia.

If untreated, it causes anemia, splenomegaly, and severe bone deformities. It progresses to death before age 20.

SIGNS AND SYMPTOMS Symptoms of anemia occur in proportion to the rate of

development of anemia; rapidly developing anemia has more profound features than slowly developing anemia. Because most patients develop anemia slowly, most have few symptoms until the condition worsens.

Usual symptoms include fatigue,palpitations, shortness of breath, abdominal pain, bone pain, tingling of fingers and toes, and muscular weakness.

Signs of anemia may include jaundice, pallor, cracking, splitting and spooning of the fingernails,increased size of the liver and spleen, lymphadenopathy, and blood in the stool. Patients with anemia may also describe a sore or painful tongue (glossitis), a smooth tongue, or redness of the tongue or cheilosis . Some patients may complain of loss of taste sensation.

Sickle cell anemia. A, Leg ulcer, and B, growth deformation of the middle finger from vaso-occlusive attackand dactylitis of the growth plate.

SCREENING LABORATORY TEST If the dentist identifies a patient with signs or symptoms

suggestive of anemia, this patient should be sent to a commercial laboratory for a complete blood count and differential, or referred to a physician for evaluation. Hb level, hematocrit, and RBC indices (mean corpuscular volume [MCV], mean corpuscular hemoglobin [MCH], and mean corpuscular hemoglobin concentration [MCHC]) are tests that are used toscreen the patient. In addition, total white blood cell (WBC) count and platelet count should be obtained todetermine whether a generalized bone marrow defect has occurred and to inspect for hypersegmentedneutrophils . Anemia is generally defined as Hb < 12 g/dL for women and < 13 g/dL for men.

In accordance with the size of RBCs, anemia is classified as microcytic (MCV < 80μm3),macrocytic (MCV > 96μm3), or normocytic (MCV, 80 mm3to 96 mm3).

A reticulocyte count lessthan 1% indicates inadequate RBC production in the bone marrow, whereas a value greater than 1%indicates increased production in response to bleeding or destruction.

ORAL MANIFESTATION Oral findings in patients with anemia usually relate to

the underlying cause of the anemia. The oral mucosa often appears pale. Patients with nutritional causes of anemia (e.g., vitamin B12 or irondeficiency) may show loss of papillae from the tongue and atrophic changes in the oral mucosa. Angular cheilitis and aphthae may be found. Patients also may report a burning or sore tongue. Some patients with iron deficiency anemia develop Plummer-Vinson syndrome ,which is characterized by a sore mouth, dysphagia (resulting from muscular degeneration in the esophagus with esophageal stenosis or “webbing”), and an increased frequency of carcinoma of the oral cavity and pharynx. Patients with this syndrome should be followed closely for any oral or pharyngeal tissue changes that might be early indicators of carcinoma

Patients with hemolytic anemia (e.g., sickle cell anemia) may show pallor and oral evidence of jaundice caused by hyperbilirubinemia caused by excessive erythrocyte destruction. The trabecular pattern of the bone on dental radiographs may be affected because of hyperplasia of marrow elements in response to increased destruction of RBCs. Therefore, dental radiographs may show enlarged bone marrow(medullary) spaces associated with bone marrow hyperplasia, increased widening and decreased numbers of trabeculations, and generalized osteoporosis (thinning of the inferior border of the mandible).Because of compensatory marrow expansion, the bone appears more radiolucent with prominent lamellar striations.

Specifically, the trabeculae between teeth may appear as horizontal rows or as a“stepladder” . This can also manifest as frontal bossing and/or “hair on end” in the cortical regions of a skull film. Vaso-occlusive events can promote asymptomatic pulpal necrosis,osteomyelitis, ischemic necrosis within the mandible, and peripheral neuropathy. Patients with sickle cell anemia often have delayed eruption of the teeth and dental .hypoplasia

ORAL MANIFESTATION THALASSEMIA The oral cavity of the beta major thalassemia

patients shows bigger upper jaw because of bone marrow expansion (chipmunk facies) and have class II div 1 malocclusion

The lower jaw is wider than a normal lower jaw . the tooth size is similar to normal child tooth

except for the multiple diastema due to faster jaw growth .

The gingiva is pale especially when the patient’s Hb drops to below 8gr/dl .The color of the gingival sometimes tends to be dark , which is caused by the high ferritin level in the blood .

Chipmunk facies, maxillary protrusion due to expansion of the facial bones, consequent to medullary space expansion and thinning of the cortical plates. Pic by Dr. Mohamad Azhar Ibrahim Kharsa, DDS, PhD. Orth http://orthocj.com/2008/01/orthodontic-characteristics-of-thalassemia-patients/

• The skull alterations are due to overactivity of the red marrow in response to anemia. It produce the hair-on-end appearance. Pic by Margaret A. Hollar, DO

• http://radiology.rsna.org/content/221/2/347.full•  

MANAGEMENT The dentist should obtain a careful history

to identify conditions associated with anemia. Inclusion of questions concerning dietary intake, malnutrition, alcohol or drug use, use of nonsteroidal anti-inflammatory drugs, menstrual blood loss, pregnancies, hypothyroidism,jaundice,gallstones,

splenectomy, bleeding disorders and abnormal Hb, and organ transplantation is important. Historical information concerning family members is also important for identifying hereditary risk for hemolytic anemias.

In children, questions should assess normal growth. When the history of a woman is taken, questions that reveal the onset, nature, and regularity of the patient's menstruation cycle may be important. Women with a history of regular periods but with heavy flow may be anemic and should receive medical advice and treatment. A patient with a change in the pattern, onset, length, or rate of menstrual flow should been couraged to seek medical evaluation. Patients who stopped having periods long before expected should be referred for medical evaluation, as should those who have had bleeding between regular periods. In addition, several historical questions should be posed to women who are pregnant or who recently experienced childbirth. For example, the dentist should establish whether the patient had excessive bleeding during pregnancy, and whether the patient has other children and when they were born, because the closer together the pregnancies were, the greater is the risk for developing iron deficiency anemia. Once the baby is born, the mother may lose additional iron during delivery and breast feeding.

The dentist should be keen to identify signs and symptoms of anemia in patients who are seen for dental treatment. A patient with classic signs or symptoms of anemia should be referred directly to a physician and screened by appropriate laboratory tests. Screening tests should include complete and differential blood counts, a smear for cell morphologic study, Hb or hematocrit count, a Sickledex test(for African Americans), and platelet count. If screening tests are ordered by the dentist and results of one or more are abnormal, the patient should be referred for medical evaluation and treatment.

Assessment of the severity of a patient's anemia is important for preventing complications. First and foremost, the dentist should ensure that the patient's underlying condition is under therapeutic control before proceeding with routine dental treatment. In many cases, anemia is associated with chronic illness; thus, treatment may be provided in the presence of anemia. For minimal medical complications,the patient's Hb should be above 11 g/dL and the patient should be free from symptoms. Patients who are short of breath and who have Hb < 11 g/dL, an abnormal heart rate, or an oxygen saturation less than 91% (as determined by pulse oximetry) are considered unstable, and routine treatment should be deferred until their health status improves.

G6PD MANAGEMENT Patients with G-6-PD deficiency have an

increased incidence of drug sensitivity, with sulfonamides(sulfamethoxazole), aspirin, and chloramphenicol being the prime offenders.

Penicillin,streptomycin, and isoniazid also have been linked to hemolysis in these patients. Dental infection may accelerate the rate of hemolysis in patients with this type of anemia.

Thus, dental infections should be avoided, and, if they occur, they must be dealt with effectively. The astute clinician will realize that febrile illness and elevated bilirubin are features of this condition. The drugs listed previously should not be used in these patients.

SICKLE CELL PATIENT MANAGEMENT Confirm with patient's physician that the condition is stable. Arrange short appointments. Avoid long and complicated procedures. Maintain good dental repair. Institute aggressive preventive dental care.

a.Oral hygiene instructionb.Diet controlc.Toothbrushing and flossingd.Fluoride gel application

Avoid oral infection; treat aggressively when present. Use pulse oximeter, maintain O2 saturation above 95%. Use local anesthetic without epinephrine for routine dental care. For surgical

procedures,use 1:100,000 epinephrine in local anesthetic. Avoid barbiturates and strong narcotics; sedation may be attained with

diazepam (Valium). Use prophylactic antibiotics for major surgical procedures. Avoid liberal use of salicylates; control pain with acetaminophen and codeine. Use nitrous oxide–oxygen with greater than 50% oxygen, high flow rate, and

goodventilation.

THALASSEMIA MANAGEMENT Dental treatment should coincide with the

hemoglobin level. Consult hematologist for dental appointment. Usually dental treatment is made after the blood transfusion is done.

Short dental appointment No dental treatment when hemoglobin is

less than 10 gm/dl Prophylactic antibiotic if patient have

undergone splenectomy Start orthodontic treatment at younger

age than normal patient

Higher risk for Hepatitis B, C, and HIV infection due to high frequency of blood transfusion

Local anesthesia is safe May be contraindicated for general

anesthesia during severe anemia state General anesthesia intubation may be

difficult due to maxilla enlargement Emphasize on preventive dentistry by

giving topical fluoride, fissure sealant and OHI

REFERENCES Dental Management of Medically

Compromised Patient 7th Edition Mosby 2007

Thalassemia and Dentistry by Abdolreza maadi DDS