HIV Encephalopathy

"More than 50% of children with HIV have neurological abnormalities including delayed motor development, loss of milestones, seizures or encephalopathy with a lack of growth and a decline in head circumference." page 233 Principles of Medicine in Africa.

Sarah watches other children play outside

Sixteen month old Sarah was much smaller than she should have been, weighing in at a mere 14 pounds. An incomplete antibiotic course had recently been given at an outside health center for presumed sepsis so I was concerned about partially treated meningitis/encephalitis. Yet, her wasting was so marked that it was clear she had a chronic not an acute condition.  Six months earlier she had had an HIV test done elsewhere but the result was not known by her parents. They reported it had never come back. Since birth she had been breastfed and her mother was not known to be HIV positive. The chief complaint on admission was "She no longer lifts her head or speaks." She was extremely thin, apathetic and anorectic, showing no interest in the world not even crying with the placement of an IV or the threat of an exam. Her mother held her head propped up. Although her head circumference was small for age, her head appeared large compared to her body. It lolled like a mushroom cap a top the stem of her neck. Her parents walked about with her draped over one shoulder, head dangling. Neurologically she seemed shockingly distant; failing to follow with her eyes, grasp a toy, sit up or make any vocalizations. The history that she had said words, smiled, and even walked seemed totally at odds with her presentation. An LP was surprisingly unremarkable for such a dramatically abnormal neurological presentation, but her HIV test was positive. ARV's and Cotrimoxazole were started. Additionally due to recurrent fevers, following a week of Ceftriaxone, our best antibiotic she was started on TB medications. After about ten days of ARV's her mother reported one day, "She smiled." No one else had seen it but Frank, the medical student, and I were pleased at some improvement, any improvement, even imaginary. By the end of two weeks, amazingly she was holding her head up and watching other children play in the courtyard. Is this the way HIV encephalopathy responds to ARV's in children? I have had no other experience to compare with this.

I recently learned in Zambia ( and I suspect elsewhere) if you don't breast feed your baby people will ask you why and suspect you have HIV/AIDS. The stigma is great and choosing not to expose your baby to HIV through breast feeding is therefor not really an option. Child survival continues to be significantly better in breast-fed infants in places where water sources are unclean and infectious diseases and malnutrition pose a significant risk of death.Yet breast-feeding contributes greatly to mother to child transmission. Strategies to reduce transmission include treating mothers and infants with ARV's during the period of breast feeding.

Yona and Gracie

Every year an estimated 12 million children die before they reach their 5th birthday. Seven out of every ten of these deaths are due to diarrhea, pneumonia, measles, malaria and malnutrition. In developing countries an estimated 50 million children under the age of five are malnourished. At least 25% of the world’s pediatric population is undernourished. Malnutrition contributes to fifty-four percent of deaths in children 0 to 4 in developing countries and is most commonly seen between 6 months and 30 months. Malnourished children are more likely to have severe even fatal as well as just longer lasting infections. Malnourishment puts children at risk for delayed mental development, behavioral problems, school performance issues and poor employment. Even a small degree of iron deficiency anemia can affect learning.

Severely malnourished children admitted to hospital face a 30-50% case fatality rate. With appropriate treatment, this high death rate can be reduced to less than 5%.The difference in mortality rate is not due merely to the level of disease severity but is related to the level of care. Severe malnutrition is a medical emergency. Hypoglycemia, hypothermia and silent infection can quickly lead to death.Children with malnutrition are often identified as having either Kwashiorkor or Marasmus. Children with Kwashiorkor appear edematous and children with Marasmus appear starved and thin. Kwashiorkor malnutrition is thought to be caused by protein deficiency and Marasmus by energy or total calorie deficiency. Kwashiorkor is the word for the disease given by the Ga tribe in Ghana meaning “the sickness the older child gets when the next baby is born” and is associated with inadequate diet during the weaning period. An inadequate diet is rich in carbohydrates but poor in proteins. In Malawi children are often given only two meals a day; corn porridge for breakfast and then later a meal primarily of nshima a powdered corn flour boiled with water into dough that is served with a relish made from greens, onion and sometimes tomatoes. Fruit and ground nuts are sometimes also given.

There are ten essential steps in the management of malnutrition
    1. Treat/prevent hypoglycemia.
    2. Treat/prevent hypothermia
    3. Treat/prevent dehydration
    4. Correct electrolyte imbalance
    5. Treat/prevent infection
    6. Correct micronutrient deficiencies
    7. Start cautious feeding
    8. Achieve catch-up growth
    9. Provide sensory stimulation and emotional support
   10. Prepare for follow-up after recovery

With one nurse for 75 patients at Nkhoma Hospital in Malawi, it is impossible to monitor all the patients regularly during the night. It is up to the guardians to alert the nurses when there are problems. Children with severe malnutrition need to be kept warm and dry. Guardians may only bring a thin cotton cloth to the hospital. Children need to be fed day and night. The guardians are provided specially formulated milk F75 and F100. It is the guardians who must take responsibility for giving the children regular feeding throughout the night. These children are especially difficult to care for as they are apathetic, anorectic, withdrawn, irritable and generally miserable. Their appearance is often abhorrent; skin is sloughing and bleeding, bodies edematous and wasted, disfigured with loss of hair and sores. Corneal ulcerations, keratomalacia, corneal scarring and irreversible are common with vitamin A deficiency. Malnutrition leads to impairment of the immune system, impaired absorption of nutrients, increased catabolism, poor appetite, vomiting, diarrhea, vitamin deficiencies, impaired skin defenses and cardiac dysfunction. The causes of malnutrition are complex and involve social, economic, cultural, and political elements. Some of these include such things as maternal deprivation and abandonment, child abuse, food taboos, poor child rearing practices, misconception about the use of certain foods, inadequate feeding, improper food distribution within family members, overcrowding and unsanitary living conditions, weather patterns, post-harvest food losses due to bad storage, natural disasters such as drought and famine, and civil conflict. Yona’s 9 month old little sister Gracie finally got sick today. 

Gracie is the reason Yona is hospitalized with Kwashiorkor. When she was born, Yona was pushed off the breast. Since then Yona has eaten mostly starchy foods and little protein. Gracie is a little underweight as she is primarily receiving breast milk. Now Gracie is sick. It is not surprising in many ways since her last two weeks have been in our ward surrounded by sickness. She has spent a lot of time on the floor of the ward eating crumbs she dropped there. She and her mother have moved among our sickest patients with pneumonia, TB, meningitis and malaria. She has been watching the F 75 NG feeds, the daily pills, the deaths of children and the wails of their parents. She has witness episodes of vomiting, diarrhea, crying, screaming, moaning and tears. She has heard countless interview questions with countless parents as we admit over 25 new patients per day. Gracie has also seen her brother improve. When Yona first came to Nkhoma Hospital his eyes were so edematous they were swollen shut. She has seen it all and now she is entering the fray more personally as we stick her for a rapid malaria test, then draw blood for her hemoglobin, take her temperature and listen to her lungs. It is pneumonia and yes, her rapid malaria test is positive. She will need an IV for quinine. Hospitals are dangerous places to hang out!

Going Home

Tadala is a 14 year old girl initially thought to have nephrotic syndrome. Nephrotic syndrome leads to massive swelling of the legs, abdomen and around the eyes. It is caused by the loss of large amounts of protein in the urine. The volume of blood is not increased and high blood pressure is not usually seen. In the developed world, nephrotic syndrome is not uncommon and is frequently treated with steroids and diuretics with good resolution. In Africa, there are many other causes for this kind of body swelling including strep infections in the throat and skin, leprosy, malaria, drugs, HIV nephropathy, connective tissue diseases such as lupus, schistosomiasis, heart failure, cirrhosis and hepatitis B.  Tadala presented with edema; puffy eyes and swollen legs and stomach disfiguring her face and making it difficult for her to walk. She was admitted to the hospital weighing 41 kgs and treatment was started with Lasix and Prednisolone for presumed nephritic syndrome. When a urinalysis was obtained however there were many red blood cells and only a small amount of protein. Additionally when I took her blood pressure it was elevated at 170/130. She was complaining of headache. It seemed she had glomerulonephritis with malignant hypertension not nephrotic syndrome. There was no history of strep or skin infection.  During the two weeks she was in the hospital she received a combination of four different blood pressure medications; Lasix, Hydralazine, Nifedipine and Aldomet to control her blood pressure. Due to electricity outages we were unable to initially obtain any blood tests. When we did they showed acute renal failure with a creatinine of 5.2 and potassium which remained below 6.5. We talked with her family about limiting bananas, restricting fluids and not salting the relish she ate with her nshima. Slowly, slowly (pong’ono pong’ono) as her blood pressure improved her kidney function began to return toward normal. Her weight dropped 7 kilograms or 15and ½ pounds. The day the edema disappeared from her legs and face she began to smile in a shy happy way whenever we looked her way. Her mother and grandmother thanked us effusively and I reminded them that we are only servants of God. We must all thank God for his faithfulness. “Mulungu akukuchizani” God has healed her! 

Joy and Sorrow

It’s six am when a child arrives from the outpatient department. I can see his lips are blue and he is gasping. Lord help me help this child. The night nurse Kate is tired. We need oxygen. Get an ambubag. We need an IV. Check the blood sugar. The blood sugar is 19. Place an NG. Give dextrose. We need an IV. Bag the child he is gasping. Others arrive. Still there is no IV. His arms and legs are icy cold. We cannot get an O2 saturation reading. His heart is still pumping. Give him IM Ceftriaxone.  Finally we push dextrose through a jugular IV and the child begins to breathe on his own. The rapid diagnostic test for malaria is positive. Start quinine. Hours later the child is crying. An LP is done. The child is sitting up in his parents’ arms and…. drinking? I am amazed. At lunch I go home and dance before the Lord-it is miraculous. The Lord literally brought this child back from the dead. Late in the afternoon, Olive tells me the child is cold and the O2 sat is not registering. She is going to get some more blankets. Dread. I feel it as I move toward the bed. How can it be? The child is gasping again. I call for help. Amanda bring the ambubag. Is it possible this miracle is over so quickly? I cannot hear heartbeats. We are giving compressions and breaths. We give dextrose but the blood glucose is fine. There is nothing more to do. The body is still warm. How can this child be dead? I cannot believe this child is gone. Joy and Sorrow in such quick succession. Death is here. It is over. I comfort the crying mother. Olive prepares the body. The parents held their child a little longer. I think of Psalm 139. This day was written in His book before there was even one. I go to the lab to look for answers the LP result- normal, the electrolytes-normal. I go home to read about cardiogenic shock. “Myocardial insufficiency occurs with ongoing hypoxic ischemic injury, alterations of intravascular volume, electrolyte disturbances, arrhythmias, shock, heart failure and cardiac arrest may occur.” Another child dies needlessly of malaria. He had no bed net. There had been no indoor residual spraying in his village. No screens on the windows. No bug spray. No prophylactic antimalarials. I think God weeps as I do.  

Elise and Boniface

Baby Elise presented to the hospital with severe anemia due to malaria with a hemoglobin of 3.5 requiring a blood transfusion (normal is 12). Following the transfusion she developed fever and began to have respiratory distress. She was grunting and hypoxic. Her liver and spleen were enlarged and a gallop rhythm alerted us to heart failure. Several doses of Furosemide (a diuretic) were given. She began urinating and her distress lessened. Still she was working hard. We gave her rectal Tylenol and sponge bathed her. I feared she might wear out. I prayed, nurse Olive prayed, her mother prayed. The family refused transfer to KCH hospital in Lilongwe which has ventilators. They did not have the money for transportation home. They would prefer to stay at Nhkoma even if Elise should perish. Was this transfusion related lung injury causing fever, hypoxemia, dyspnea and hypotension or fluid overload in a severely anemic child with heart failure? It is hard to sleep at night when you are caring for critically ill children with very few resources. I prayed Psalm 72 for Elise. “He will deliver the needy when he cries for help. The afflicted also. He will have compassion on the poor and needy and the lives of the needy he will save. He will rescue their life…and their blood will be precious in his sight.” The next morning Elise was sitting up playing on her mother’s lap. Her mother and I thanked God together.

I want all the children to survive and to be completely restored to health. The children who survive cerebral malaria are among the most severely neurologically damaged and the least likely to be restored to wholeness. They have often seized for hours and been hypoxic during that time prior to arriving at the hospital. Boniface was having decorticate posturing the first day that I examined him. At first I thought he was still seizing he was so rigid. His head was arched backwards, his leg muscles rigid with his legs held out straight and hands tightly fisted with arms inwardly turned. His eyes were deviated to the left. Could he have had an intracranial hemorrhage? Was this encephalopathy and hypoxia? For days he lay in his teenage mother’s arms unresponsive. I cannot imagine the future for this mother and child. If he does not suck how will he survive? How will she feed him? I encourage her to keep on pumping so her milk will not dry up as we feed the child by an NG. Can they place NG tubes in the village and feed him the way that we are? I pray every day for this child. Gradually his mother begins reporting that Boniface is making some effort at nursing. It seems unlikely as I watched them trying to spoon a little porridge in his mouth and wonder if I should stop them in case he aspirates. The Physical Therapy department comes by to teach the mother how to move his arms and legs to prevent contractures. We stop the anticonvulsants. Pong’ono pong’ono (little by little) he becomes more alert. He cries when uncomfortable. Then one day, his hands are no longer fisted and his tone is decreased. He appears to briefly fixate on an object moving in front of his face. He is nursing deliberately and strongly. I thank God and I keep on praying and hoping for little Boniface.

 

Limited Resources

At Nkhoma Hospital, Malawi and many hospitals in developing countries, resources such as clean water and elcetricity can be intermittent or nonexistant affecting patient care dramatically. Although there are often back up generators and water in a bore hole which can be carried, the challenges are profound.

ELECTRICITY OUT

Electricity out -oxygen stops,

Electricity out- warning beeps,

Saturations drop, heart rates rise,  

Electricity out- no ultrasound,

Electricity out- computers surge and stop,

Electricity out-charts and children plunge into darkness,

A voice in the dark calling Ali Bwanji "How is he? How is she?

The child can be made out scarcely in the dim light of dawn,

Holding the chart up to the unscreened window,

Trying to make out the orders:

    the medications

          the hemoglobin result

                 the malaria smear

                        the temperature

                                the weight

Listening to this child whimpering  above the general  crying of children on the ward

Electricity out-spiritual warfare

Electricity out-battle cry

 

Cerebral Malaria

Cerebral Malaria is a serious complication defined by coma, convulsions and encephalopathy which can lead to death.

          Yona

Sick two days, fever, diarrhea,

Playing, robust, chubby legs, warm heart, smiling,

Eating porridge in the morning.

Convulsions, coma, diazepam,

Last breaths, strong heart,

Ambu bag pushing breaths in and out,

NG tube placed, stomach decompressed.

Mother waiting watchful,

dreading, hopeful,

hopeless,

1 hour, 2 hours, 3 hours,

When to stop- it is my call,

My wild God is in charge,

He knows the why.

Eyes fixed and dilated,

Let him go quietly.

His mother waits on the floor across the room,

A torn hole in her light blue sweatshirt, blood on her skirt- her child's? her own?

Strong heart. The nurses would have gone on breathing for him for hours,

They were not tired.

Decorticate, decerebrate, brain dead, eyes fixed and dilated,

My wild God loves Yona and He  loves me. Lord, comfort his mother in her pain

and loss.  You understand.

Your son died too.

Jesus,  "a man of sorrows and familiar with sufffering."

Give her strength to bear this grief.

reference: Isaiah 53:3, 4