Georgians with sickle cell disease lack the care and attention they need
September was Sickle Cell Awareness Month. It’s now October, and Georgia still has two numbers worth sitting with.
Daily preventive penicillin cuts life-threatening infection in young children with sickle cell anemia by roughly 84%. It is cheap, old and simple.
In the decade to 2019, 15.5% of Georgia children who should have been on it filled prescriptions covering at least 300 days of the year. In California, 22.2%.
The second number stays with me. An annual ultrasound identifies which children are at high risk of stroke. A child with an abnormal scan faces a 40% risk of stroke within three years.
Begin regular transfusions in those children, and that risk falls by 92%. Georgia screened 52.7% of the children who should have been screened in those years; California, 47.4%. Georgia did better; both missed about half.
So, we have a test that finds the children at highest risk, a treatment that prevents nine in 10, and we run the test on half.
We know this only because someone counted.
There is no endorsed quality measure for adults
America has developed exactly two nationally endorsed quality measures for sickle cell disease, and you have read both of them scored. One covers preventive antibiotics in children aged 3 months to 5 years. The other covers that annual ultrasound in children aged 2 to 15. That is the entire list.
Both stop in childhood. One stops at 5.
There is no endorsed quality measure, none, for a single adult in this country living with sickle cell disease, which is where the disease does its worst work.
More than half of Americans with it rely on Medicaid or CHIP. Among those, 78% went to an emergency department in 2017, and 49% were hospitalized. Life expectancy runs more than 21 years short.
And the two measures we do have, no one is obliged to report. An expert panel convened for the federal government recommended adding them to the set states report on. For 2019, Washington declined.
In May, Georgia Gov. Brian Kemp signed House Bill 334. It requires the Department of Community Health to review every year whether what Georgia Medicaid covers is, in the law’s words, adequate to meet the needs of Medicaid recipients with a diagnosis of sickle cell disease. It must seek public input, report to the General Assembly by Jan. 15, and publish that report online.
Read that again. Adequate to meet the needs. You cannot judge adequacy from a coverage list.
Georgia Medicaid covers penicillin. 15.5% of the children who needed it got it. That is a finding about adequacy, and the state already has the number.
Three recommendations to make the report more valuable
The first report under HB 334 is due in January. Three additions would make it a model.
- First, put the numbers in it. They belong in a report about adequacy.
- Second, ask who can deliver the care. Hematologists are scarce: of 134 fellowships, 132 are joint with oncology. Two-thirds of primary care physicians surveyed said they were not well informed about the screening guidelines; 6% of hematologists said so. The National Academies concluded primary care clinicians could manage this care with the right preparation. The shortage is not of doctors. It is of prepared ones.
- Third, build past 15. Experts have agreed on indicators for the handover to adult care: whether the two doctors ever spoke, how long the young adult waited for her first appointment, whether she trusts her new doctor. Nobody collects them routinely. Georgia could lead.
One last thing, for families rather than the state. Before the department writes that report, the law requires it to ask people who know this disease what they need, and to consider the answers. No one is required to give one. However the state asks, by hearing, survey or form, answer. Families came to the Capitol to pass this law. This will feel smaller. It counts the same.
Awareness months ask for sympathy. The month after should ask for arithmetic.
The families I have met do not need to be told their disease is serious. They have known since the newborn screen. What they need is for the rest of us to keep track, past 5, past 15, past the age at which our attention expires.
The disease does not stop at 15. Only the counting does. Georgia has given itself somewhere to say so.
Michael V. Smith, MD, is a board-certified cardiothoracic surgeon and founder and chief executive of Marti Health, which cares for Georgians living with sickle cell disease under contract with the state Medicaid program.
Disclosure: Marti Health, Inc. holds a contract with the Georgia Department of Community Health for care management of a sickle cell cohort.
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